Medicine › Pathology and Forensic Medicine

Autoimmune Bullous Skin Diseases

Works Count: 60831

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Description

This cluster of papers focuses on the pathogenesis, diagnosis, and treatment of pemphigoid diseases, particularly bullous pemphigoid and pemphigus vulgaris. It covers topics such as autoantibodies, rituximab therapy, epidemiology, and the use of immunosuppressive therapy including intravenous immunoglobulin. The research also delves into the role of desmoglein in disease pathology and autoimmune mechanisms.

Keywords

Pemphigoid; Autoantibodies; Rituximab; Bullous Pemphigoid; Desmoglein; Autoimmune; Immunosuppressive Therapy; Diagnosis; Epidemiology; Intravenous Immunoglobulin

Bullous pemphigoid and herpes gestationis autoantibodies recognize a common non-collagenous site on the BP180 ectodomain.

1993-11-15

George J. Giudice , Derek Emery , Brian D. Zelickson +3 more

Abstract Bullous pemphigoid (BP) and herpes gestationis (HG) are skin diseases characterized by subepidermal blisters and autoantibodies against two hemidesmosomal Ag, i.e., BP230 and BP180. Based on sequence analysis the … Abstract Bullous pemphigoid (BP) and herpes gestationis (HG) are skin diseases characterized by subepidermal blisters and autoantibodies against two hemidesmosomal Ag, i.e., BP230 and BP180. Based on sequence analysis the BP180 Ag was predicted to be a transmembrane protein with a long extracellular collagenous domain. In the present investigation fusion proteins encompassing various segments of the BP180 Ag were expressed in a prokaryotic system and assayed by immunoblotting and immunoadsorption against a panel of BP, HG and control sera. One antigenic site, comprising 14 amino acids of the BP180 noncollagenous (NC) 16A domain, was shown to be recognized by 60% of BP sera and by 63% of HG sera tested. 73% (11/15) of BP sera and 100% (8/8) of HG sera reacted with at least one of three BP180 fusion proteins representing various portions of the NC16A domain. Immunoadsorption analysis identified this region of BP180 as an immunodominant site. Using an affinity purified rabbit antiserum raised against a recombinant form of BP180, this BP/HG autoantibody-reactive region was localized to the epidermal basal lamina immediately adjacent to the hemidesmosome. These findings confirmed the predicted type II transmembrane orientation of the BP180 Ag. Thus, the long, C-terminal collagenous domain of this basal keratinocyte protein projects into the basal lamina and may function as a site of interaction with an extracellular matrix component. It is proposed that autoantibodies directed against the well-defined antigenic site on the BP180 ectodomain may play an initiatory role in subepidermal blister formation in BP and HG patients.

Toxin in bullous impetigo and staphylococcal scalded-skin syndrome targets desmoglein 1

2000-11-01

Masayuki Amagai , Norihisa Matsuyoshi , Zhi Hong Wang +2 more

Molecular heterogeneity of the bullous pemphigoid antigens as detected by immunoblotting.

1986-02-15

Ramzy S. Labib , Grant J. Anhalt , Harish Patel +2 more

Sera from 28 patients with bullous pemphigoid (BP), four patients with cicatricial pemphigoid (CP), and 24 controls (normal volunteers and patients with pemphigus, systemic lupus erythematosus, or other skin diseases) … Sera from 28 patients with bullous pemphigoid (BP), four patients with cicatricial pemphigoid (CP), and 24 controls (normal volunteers and patients with pemphigus, systemic lupus erythematosus, or other skin diseases) were tested against extracts of human epidermis by immunoblotting techniques. The extraction buffer included 1% SDS, 5% beta-mercaptoethanol, and six protease inhibitors with various specificities. BP sera from individual patients showed different patterns of reactivity with the same epidermal extract, and each pattern consisted of one or more bands. A total of five bands of 240 kD, 200 kD, 180 kD, 97 kD, and 77 kD reacted with BP sera; the 240-kD band reacted with one CP sera, and none of these bands was detected by the control sera. The 240-kD and 180-kD bands reacted very strongly with some sera and were most frequently observed (43% and 29%, respectively). The 200-kD, 97-kD, and 77-kD bands were less frequently observed (25%, 7%, and 7%, respectively), but when present, their reactions were usually strong. Eleven percent of the BP sera did not react with any bands. Contrary to previous reports, this study shows that BP autoantibodies react with several protein bands, as detected by immunoblotting. We have recently shown by immunoelectron microscopy that BP autoantibodies bind to the basal cell hemidesmosomes. It remains to be determined which of these protein bands represent specific hemidesmosomal proteins and which antibody-antigen interactions are relevant to the pathogenesis of this disease.

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Characterization of autoantibodies in pemphigus using antigen-specific enzyme-linked immunosorbent assays with baculovirus-expressed recombinant desmogleins.

1997-08-15

Ken Ishii , Masayuki Amagai , Russell P. Hall +5 more

Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune skin diseases caused by autoantibodies against desmoglein (Dsg) 3 and Dsg1, respectively. Routine immunofluorescence testing of skin and serum from patients … Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune skin diseases caused by autoantibodies against desmoglein (Dsg) 3 and Dsg1, respectively. Routine immunofluorescence testing of skin and serum from patients cannot distinguish between these two severe diseases since both have IgG Abs directed against keratinocyte cell surfaces. In this study, recombinant Dsg3 and Dsg1, produced as secreted proteins by baculovirus expression, have been utilized to develop ELISAs for the specific characterization of their autoantibodies. Of 49 PV sera, 46 were positive in the Dsg3 ELISA and 44 of 46 PF sera were positive in the Dsg1 ELISA, compared with only 3 of 23 sera of bullous pemphigoid, and none of 53 normal control sera in both ELISAs. Both the Dsg3 and Dsg1 ELISAs were more specific and sensitive than conventional immunofluorescence staining. These Ag-specific ELISAs revealed that more than one-half of PV sera (26 of 49) had anti-Dsg1 Abs in addition to anti-Dsg3 Abs. PV patients who had not only oral mucous lesions but also significant skin involvement tended to have higher titers of anti-Dsg1 Abs. Furthermore, the ELISA reactivity correlated well with clinical disease activity in 5 of 6 PV and 5 of 5 PF patients. This ELISA provides a sensitive and highly specific assay for the diagnosis of patients with PV and PF, the correlation of disease activity with serum Ab levels, and a novel tool for investigating the immunopathogenesis of pemphigus.

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Anti‐p200 pemphigoid: A novel autoimmune subepidermal blistering disease

2006-12-20

Amrei Dilling , Christian Rosé , Takashi Hashimoto +2 more

ABSTRACT Anti‐p200 pemphigoid is a recently defined autoimmune subepidermal blistering disease characterized by circulating and tissue‐bound autoantibodies to a 200‐kDa protein (p200) of the dermal–epidermal junction (DEJ). This DEJ constituent … ABSTRACT Anti‐p200 pemphigoid is a recently defined autoimmune subepidermal blistering disease characterized by circulating and tissue‐bound autoantibodies to a 200‐kDa protein (p200) of the dermal–epidermal junction (DEJ). This DEJ constituent is thought to be important for adhesion of basal keratinocytes to the underlying dermis. While the exact identity of p200 remains unknown, it has been demonstrated to be immunologically and biochemically distinct from all major autoantigens of the DEJ, including bullous pemphigoid antigens 180 and 230, laminin 1, 5 and 6, α6β4 integrin, and type VII collagen. Clinically, most reported cases present with tense blisters as well as urticarial papules and plaques, closely resembling bullous pemphigoid. Histopathological examination of lesional skin biopsies shows subepidermal split formation and superficial inflammatory infiltrate typically dominated by neutrophils. Immunopathologically, linear deposits of immunoglobulin (Ig)G and C3 are detected along the DEJ by direct immunofluorescence microscopy of perilesional skin. Indirect immunofluorescence microscopy of patients’ sera on NaCl‐split human skin demonstrates circulating IgG autoantibodies labeling the dermal side of the split. By immunoblotting, these autoantibodies recognize a 200‐kDa protein of human dermis. Biochemical characterization of the p200 molecule revealed a noncollagenous N‐glycosylated acidic protein with an isoelectric point of approximately 5.5. We present an overview of the pathogenesis, clinical features, diagnosis and treatment of this new disease entity.

The clinical phenotype of pemphigus is defined by the anti-desmoglein autoantibody profile

1999-02-01

Masayuki Amagai , Kazuyuki Tsunoda , Detlef Zillikens +2 more

A comparison of current acne grading systems and proposal of a novel system

1997-06-01

Amol Doshi , Ahmed Zaheer , Matthew J. Stiller

Cloning and Primary Structural Analysis of the Bullous Pemphigoid Autoantigen BP180

1992-09-01

George J. Giudice , Daryl J Emery , Luis A. Diaz

DISEASES OF THE SKIN

1921-11-01

OLIVER S. ORMSBY

Characterization of bullous pemphigoid antigen: A unique basement membrane protein of stratified squamous epithelia

1981-06-01

John R. Stanley , Pamela Hawley‐Nelson , Stuart H. Yuspa +2 more

Induction of Pemphigus in Neonatal Mice by Passive Transfer of IgG from Patients with the Disease

1982-05-20

Grant J. Anhalt , Ramzy S. Labib , John J. Voorhees +2 more

We examined the role of circulating autoantibodies in the pathogenesis of pemphigus vulgaris by passively transferring IgG fractions from five patients with pemphigus vulgaris into neonatal Balb/c mice, in doses … We examined the role of circulating autoantibodies in the pathogenesis of pemphigus vulgaris by passively transferring IgG fractions from five patients with pemphigus vulgaris into neonatal Balb/c mice, in doses of 1.5 to 16 mg per gram of body weight per day. Cutaneous blisters and erosions with the histologic, ultrastructural, and immunofluorescence features of pemphigus occurred in 39 of 55 mice given intraperitoneal injections of IgG from patients with pemphigus and in none of 58 control mice given normal human IgG. IgG fractions with high titers of pemphigus antibodies were most effective in inducing disease, and this effect was dose dependent. Titers of circulating IgG in mouse serum closely correlated with the extent of disease induced (P<0.002). This study strongly supports the proposed role of pemphigus autoantibodies in the pathogenesis of pemphigus vulgaris in human beings and demonstrates that pemphigus can be passively transferred to laboratory animals. (N Engl J Med. 1982; 306:1189–96.)

Differentiating Anti-Lamina Lucida and Anti-Sublamina Densa Anti-BMZ Antibodies by Indirect Immunofluorescence on 1.0 M Sodium Chloride-Separated Skin

1984-02-01

W. Ray Gammon , Robert A. Briggaman , Alfred O. Inman +2 more

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Basement Zone Antibodies in Bullous Pemphigoid

1967-05-29

Robert E. Jordon

Indirect immunofluorescent (IF) staining revealed that in 14 of 16 patients with active lesions of bullous pemphigoid the sera contained antibodies specific for the basement zone beneath stratified squamous epithelium … Indirect immunofluorescent (IF) staining revealed that in 14 of 16 patients with active lesions of bullous pemphigoid the sera contained antibodies specific for the basement zone beneath stratified squamous epithelium while the sera from six patients in remission contained no demonstrable antibodies. The sera of 18 patients with dermatitis herpetiformis and 94 patients with various bullous and nonbullous dermatoses yielded negative reactions to the basement zone by indirect IF staining. In four of five skin biopsy specimens from patients with bullous pemphigoid direct IF staining indicated that γ-globulin had been bound in vivo to the basement zone.

A Single Cycle of Rituximab for the Treatment of Severe Pemphigus

2007-08-08

P. Joly , Hugo Mouquet , Jean‐Claude Roujeau +7 more

The combination of multiple cycles of rituximab and intravenous immune globulins has been reported to be effective in patients with severe pemphigus. The aim of this study was to assess … The combination of multiple cycles of rituximab and intravenous immune globulins has been reported to be effective in patients with severe pemphigus. The aim of this study was to assess the efficacy of a single cycle of rituximab in severe types of pemphigus.

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Rituximab therapy leads to rapid decline of serum IgG4 levels and prompt clinical improvement in IgG4‐related systemic disease

2010-02-26

Arezou Khosroshahi , Donald B. Bloch , Vikram Deshpande +1 more

Patients with IgG4-related systemic disease (IgG4-RSD) frequently show an incomplete response to treatment with glucocorticoids and traditional disease-modifying antirheumatic drugs (DMARDs). B lymphocyte depletion is a therapeutic strategy known to … Patients with IgG4-related systemic disease (IgG4-RSD) frequently show an incomplete response to treatment with glucocorticoids and traditional disease-modifying antirheumatic drugs (DMARDs). B lymphocyte depletion is a therapeutic strategy known to be effective for pemphigus vulgaris, an autoimmune condition mediated by IgG4 autoantibodies. This study was performed to assess the clinical and serologic responses to B lymphocyte depletion therapy with rituximab in patients with IgG4-RSD.Four patients with IgG4-RSD were treated with 2 intravenous doses (1 gram each) of rituximab. Clinical improvement was assessed by monitoring the tapering/discontinuation of prednisone and DMARDs, and by measuring the serum concentrations of B lymphocytes, immunoglobulins, and IgG subclasses before and after therapy.Clinical features of IgG4-RSD in these 4 patients included autoimmune pancreatitis, sclerosing cholangitis, lymphoplasmacytic aortitis, salivary gland involvement, orbital pseudotumor, and lacrimal gland enlargement. The 3 patients with elevated serum IgG and IgG4 levels at baseline had a mean IgG concentration of 2,003 mg/dl (normal range 600-1,500 mg/dl) and a mean IgG4 concentration of 2,160 mg/dl (normal range 8-140 mg/dl). Among these patients, the serum IgG4 concentrations declined by a mean of 65% within 2 months of rituximab administration. All 4 patients demonstrated striking clinical improvement within 1 month of the initiation of rituximab therapy, and tapering or discontinuation of their treatment with prednisone and DMARDs was achieved in all 4 patients. A decrease in IgG concentration was observed for the IgG4 subclass only.Treatment with rituximab led to prompt clinical and serologic improvement in these patients with refractory IgG4-RSD, and is a viable treatment option for this condition. The decline in serum IgG4 concentrations was substantially steeper than that of the autoantibody concentrations in immune-mediated conditions in which rituximab is effective, such as in rheumatoid arthritis. In addition, the reduction in IgG-subclass levels appeared to be specific for IgG4. The swift improvement of IgG4-RSD suggests that rituximab achieves its effects in IgG4-RSD by depleting the pool of B lymphocytes that replenish short-lived IgG4-secreting plasma cells.

A passive transfer model of the organ-specific autoimmune disease, bullous pemphigoid, using antibodies generated against the hemidesmosomal antigen, BP180.

1993-11-01

Zhi Liu , Luis A. Díaz , James L. Troy +4 more

Subepidermal blistering associated with the human skin diseases bullous pemphigoid and herpes gestationis has been thought to be an IgG autoantibody-mediated process; however, previous attempts to demonstrate the pathogenicity of … Subepidermal blistering associated with the human skin diseases bullous pemphigoid and herpes gestationis has been thought to be an IgG autoantibody-mediated process; however, previous attempts to demonstrate the pathogenicity of patient autoantibodies have been unsuccessful. An immunodominant and potentially pathogenic epitope associated with these blistering diseases has recently been mapped to the extracellular domain of a human epidermal antigen, BP180. Patient autoantibodies that react with this well-defined antigenic site failed to crossreact with the murine form of this autoantigen and thus could not be assayed for pathogenicity in a conventional passive transfer mouse model. As an alternative, rabbit polyclonal antibodies were generated against a segment of the murine BP180 protein homologous with the human BP180 autoantibody-reactive site and were passively transferred into neonatal BALB/c mice. The injected animals developed a subepidermal blistering disease that closely mimicked bullous pemphigoid and herpes gestationis at the clinical, histological, and immunological levels. Autoantibodies that recognize the human BP180 ectodomain are therefore likely to play an initiatory role in the pathogenesis of bullous pemphigoid and herpes gestationis.

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Pemphigus and pemphigoid

1979-07-01

Walter F. Lever

Paraneoplastic Pemphigus

1990-12-20

Grant J. Anhalt , SooChan Kim , John R. Stanley +7 more

We describe five patients with underlying neoplasms in whom painful mucosal ulcerations and polymorphous skin lesions developed, usually with progression to blistering eruptions on the trunk and extremities. Histologic examination … We describe five patients with underlying neoplasms in whom painful mucosal ulcerations and polymorphous skin lesions developed, usually with progression to blistering eruptions on the trunk and extremities. Histologic examination showed vacuolization of epidermal basal cells, keratinocyte necrosis, and acantholysis. Immunofluorescence testing revealed atypical pemphigus-like autoantibodies in perilesional epithelium and serum from all five patients. We studied the antigenic specificities of the autoantibodies by indirect immunofluorescence and immunoprecipitation, using extracts of 14C-labeled human keratinocytes. IgG purified from the serum of one patient was passively transferred to four neonatal mice to test for pathogenicity.

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Development of ELISA for the specific determination of autoantibodies against envoplakin and periplakin in paraneoplastic pemphigus

2009-09-07

Christian Probst , Wolfgang Schlumberger , W. Stöcker +6 more

A Comparison of Oral and Topical Corticosteroids in Patients with Bullous Pemphigoid

2002-01-31

P. Joly , Jean‐Claude Roujeau , Jacques Bénichou +7 more

Bullous pemphigoid is the most common autoimmune blistering skin disease of the elderly. Because elderly people have low tolerance for standard regimens of oral corticosteroids, we studied whether highly potent … Bullous pemphigoid is the most common autoimmune blistering skin disease of the elderly. Because elderly people have low tolerance for standard regimens of oral corticosteroids, we studied whether highly potent topical corticosteroids could decrease mortality while controlling disease.

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Anti-laminin gamma-1 pemphigoid

2009-02-07

Teruki Dainichi , Sadamu Kurono , Bungo Ohyama +7 more

Anti-p200 pemphigoid has been characterized by autoantibodies to an unidentified 200-kDa protein (p200) of the dermal−epidermal junction. The objective of this study was to identify p200. We performed 2D gel … Anti-p200 pemphigoid has been characterized by autoantibodies to an unidentified 200-kDa protein (p200) of the dermal−epidermal junction. The objective of this study was to identify p200. We performed 2D gel electrophoresis of dermal extracts and immunoblotting with patients' sera, followed by MS analysis of a unique protein band. The protein band corresponded to laminin γ1. Anti-laminin γ1 mAb reacted with the anti-p200 immunoprecipitates by immunoblotting. Sera from 32 patients with anti-p200 pemphigoid showed 90% reactivity to the recombinant products of laminin γ1. None of the healthy control sera reacted with laminin γ1. By immunoblotting, reactivity of a patient's serum with p200 was competitively inhibited by adding anti-laminin γ1 C -terminus mAb. Purified anti-p200 IgG also inhibited the reactivity of this mAb to dermal laminin γ1. Most laminin γ1-positive sera showed reactivity with recombinant laminin γ1 C -terminal E8 fragment. Reactivity of patients' sera and purified IgG to dermal laminin γ1 was higher than reactivity to blood vessel laminin γ1 under reducing conditions. These results suggest that laminin γ1 is the autoantigen for patients with anti-p200 pemphigoid. The autoantibodies may specifically recognize dermal laminin γ1 with unique posttranslational modifications. The epitope is localized to the 246 C -terminal amino acids within the coiled-coil domain. The 9 C -terminal residues are known to be critically involved in laminin recognition by integrins.

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Autoantibodies against a novel epithelial cadherin in pemphigus vulgaris, a disease of cell adhesion

1991-11-01

Masayuki Amagai , Vera Klaus-Kovtun , John R. Stanley

Incidence and Distribution of Subepidermal Autoimmune Bullous Skin Diseases in Three French Regions

1995-01-01

Philippe Bernard

<h3>Background and Design:</h3> The incidence and distribution of autoimmune subepidermal bullous diseases were estimated from prospective data (including immunoelectron microscopy) obtained from 100 cases during a mean period of 35 … <h3>Background and Design:</h3> The incidence and distribution of autoimmune subepidermal bullous diseases were estimated from prospective data (including immunoelectron microscopy) obtained from 100 cases during a mean period of 35 months in three university dermatologic centers in Amiens, Limoges, and Tours, France, that correspond to a cumulative reference population of 3.55×10<sup>6</sup>. <h3>Results:</h3> Using data from these regions, we found a mean annual incidence of autoimmune subepidermal bullous diseases to be 10.4 per million people and, therefore, estimated the overall number of new cases of these disorders in France to be about 590 cases per year. According to clinical and immunoelectron microscopic criteria, a precise diagnosis was established in 94 cases, distributed as follows: bullous pemphigoid, 69 cases; cicatricial pemphigoid, 12 cases; linear IgA dermatosis, five cases; herpes gestationis, four cases; epidermolysis bullosa acquisita, two cases; and vesiculobullous systemic lupus erythematosus, two cases. <h3>Conclusion:</h3> Our prospective study is the first assessing the incidence and distribution of autoimmune subepidermal bullous disorders that systematically included immunoelectron microscopic data. Our estimated incidence of bullous pemphigoid (seven new cases per million people per year) is large enough to establish bullous pemphigoid as the major autoimmune subepidermal bullous disease for the purpose of therapeutic trials. On the contrary, all other disorders, particularly epidermolysis bullosa acquisita (estimated annual incidence, 0.17 to 0.26 per million people), were very rare and reflect the paucity of patients available for shortterm clinical studies in France. (Arch Dermatol. 1995;131:48-52)

Immunoglobulin G4: an odd antibody

2009-02-16

Rob C. Aalberse , Steven O. Stapel , Janine Schuurman +1 more

Despite its well-known association with IgE-mediated allergy, IgG4 antibodies still have several poorly understood characteristics. IgG4 is a very dynamic antibody: the antibody is involved in a continuous process of … Despite its well-known association with IgE-mediated allergy, IgG4 antibodies still have several poorly understood characteristics. IgG4 is a very dynamic antibody: the antibody is involved in a continuous process of half-molecules (i.e. a heavy and attached light-chain) exchange. This process, also referred to as 'Fab-arm exchange', results usually in asymmetric antibodies with two different antigen-combining sites. While these antibodies are hetero- bivalent, they will behave as monovalent antibodies in most situations. Another aspect of IgG4, still poorly understood, is its tendency to mimic IgG rheumatoid factor (RF) activity by interacting with IgG on a solid support. In contrast to conventional RF, which binds via its variable domains, the activity of IgG4 is located in its constant domains. This is potentially a source of false positives in IgG4 antibody assay results. Because regulation of IgG4 production is dependent on help by T-helper type 2 (Th2) cells, the IgG4 response is largely restricted to non-microbial antigens. This Th2-dependency associates the IgG4 and IgE responses. Another typical feature in the immune regulation of IgG4 is its tendency to appear only after prolonged immunization. In the context of IgE-mediated allergy, the appearance of IgG4 antibodies is usually associated with a decrease in symptoms. This is likely to be due, at least in part, to an allergen-blocking effect at the mast cell level and/or at the level of the antigen-presenting cell (preventing IgE-facilitated activation of T cells). In addition, the favourable association reflects the enhanced production of IL-10 and other anti-inflammatory cytokines, which drive the production of IgG4. While in general, IgG4 is being associated with non-activating characteristics, in some situations IgG4 antibodies have an association with pathology. Two striking examples are pemphigoid diseases and sclerosing diseases such as autoimmune pancreatitis. The mechanistic basis for the association of IgG4 with these diseases is still enigmatic. However, the association with sclerosing diseases may reflect an excessive production of anti-inflammatory cytokines triggering an overwhelming expansion of IgG4-producing plasma cells. The bottom line for allergy diagnosis: IgG4 by itself is unlikely to be a cause of allergic symptoms. In general, the presence of allergen-specific IgG4 indicates that anti-inflammatory, tolerance-inducing mechanisms have been activated. The existence of the IgG4 subclass, its up-regulation by anti-inflammatory factors and its own anti-inflammatory characteristics may help the immune system to dampen inappropriate inflammatory reactions.

The First International Consensus on Mucous Membrane Pemphigoid

2002-03-01

Lawrence S. Chan , A. Razzaque Ahmed , Grant J. Anhalt +7 more

We aimed to develop consensus-based recommendations for streamlining medical communication among various health care professionals, to improve accuracy of diagnosis and treatment, and to facilitate future investigations for mucous membrane … We aimed to develop consensus-based recommendations for streamlining medical communication among various health care professionals, to improve accuracy of diagnosis and treatment, and to facilitate future investigations for mucous membrane pemphigoid.Because of the highly specific nature of this group of diseases, the 26 invited participants included either international scholars in the field of mucous membrane pemphigoid or experts in cutaneous pharmacology representing the 3 medical disciplines ophthalmology, oral medicine, and dermatology.The first author (L.S.C.) conducted a literature search. Based on the information obtained, international experts who had contributed to the literature in the clinical care, diagnosis, and laboratory investigation for mucous membrane pemphigoid were invited to participate in a consensus meeting aimed at developing a consensus statement.A consensus meeting was convened and conducted on May 10, 1999, in Chicago, Ill, to discuss the relevant issues. The first author drafted the statement based on the consensus developed at the meeting and the participants' written comments. The draft was submitted to all participants for 3 separate rounds of review, and disagreements were reconciled based on literature evidence. The third and final statement incorporated all relevant evidence obtained in the literature search and the consensus developed by the participants. The final statement was approved and endorsed by all 26 participants.Specific consensus-based recommendations were made regarding the definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators for mucous membrane pemphigoid. A system of standard reporting for these patients was proposed to facilitate a uniform data collection.

Bullous pemphigoid and pemphigus vulgaris--incidence and mortality in the UK: population based cohort study

2008-07-09

Sinéad Langan , Liam Smeeth , Ruth E. Hubbard +3 more

<b>Objective</b> To determine the incidence of and mortality from bullous pemphigoid and pemphigus vulgaris in the United Kingdom. <b>Design</b> Retrospective historical cohort study. <b>Setting</b> Computerised medical records from the health … <b>Objective</b> To determine the incidence of and mortality from bullous pemphigoid and pemphigus vulgaris in the United Kingdom. <b>Design</b> Retrospective historical cohort study. <b>Setting</b> Computerised medical records from the health improvement network, a large population based UK general practice database. <b>Participants</b> Patients with pemphigus vulgaris and bullous pemphigoid diagnostic codes and age, sex, and practice matched controls. <b>Main outcome measures</b> Incidence and mortality compared with the control population by calendar period, age group, sex, geographical region, and degree of social deprivation. <b>Results</b> 869 people with bullous pemphigoid and 138 people with pemphigus vulgaris were identified. The median age at presentation for bullous pemphigoid was 80 (range 23-102) years, and 534 (61%) patients were female. The median age at presentation for pemphigus vulgaris was 71 (21-102) years, and 91 (66%) patients were female. Incidences of bullous pemphigoid and pemphigus vulgaris were 4.3 (95% confidence interval 4.0 to 4.6) and 0.7 (0.6 to 0.8) per 100 000 person years. The incidence of bullous pemphigoid increased over time; the average yearly increase was 17% (incidence rate ratio=1.2, 95% confidence interval 1.1 to 1.2). An average yearly increase in incidence of pemphigus vulgaris of 11% (incidence rate ratio=1.1, 1.0 to 1.2) occurred. The risk of death for patients with bullous pemphigoid was twice as great as for controls (adjusted hazard ratio=2.3, 95% confidence interval 2.0 to 2.7). For pemphigus vulgaris, the risk of death was three times greater than for controls (adjusted hazard ratio=3.3, 2.2 to 5.2). <b>Conclusions</b> Incidences of bullous pemphigoid and pemphigus vulgaris are increasing. The reasons for the changes in incidence are not clearly understood but have implications for identifying causative factors. Both disorders are associated with a high risk of death. Previous estimates may have underestimated the risk of death associated with these diseases.

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Explanations for the clinical and microscopic localization of lesions in pemphigus foliaceus and vulgaris

1999-02-15

Mỹ G. Mahoney , Zhihong Wang , Kyle Rothenberger +3 more

Patients with pemphigus foliaceus (PF) have blisters on skin, but not mucous membranes, whereas patients with pemphigus vulgaris (PV) develop blisters on mucous membranes and/or skin. PF and PV blisters … Patients with pemphigus foliaceus (PF) have blisters on skin, but not mucous membranes, whereas patients with pemphigus vulgaris (PV) develop blisters on mucous membranes and/or skin. PF and PV blisters are due to loss of keratinocyte cell–cell adhesion in the superficial and deep epidermis, respectively. PF autoantibodies are directed against desmoglein (Dsg) 1; PV autoantibodies bind Dsg3 or both Dsg3 and Dsg1. In this study, we test the hypothesis that coexpression of Dsg1 and Dsg3 in keratinocytes protects against pathology due to antibody-induced dysfunction of either one alone. Using passive transfer of pemphigus IgG to normal and DSG3null neonatal mice, we show that in the areas of epidermis and mucous membrane that coexpress Dsg1 and Dsg3, antibodies against either desmoglein alone do not cause spontaneous blisters, but antibodies against both do. In areas (such as superficial epidermis of normal mice) where Dsg1 without Dsg3 is expressed, anti-Dsg1 antibodies alone can cause blisters. Thus, the anti-desmoglein antibody profiles in pemphigus sera and the normal tissue distributions of Dsg1 and Dsg3 determine the sites of blister formation. These studies suggest that pemphigus autoantibodies inhibit the adhesive function of desmoglein proteins, and demonstrate that either Dsg1 or Dsg3 alone is sufficient to maintain keratinocyte adhesion.

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GENERALIZED PUSTULAR PSORIASIS.

1968-12-01

Harvey Baker , Terence J. Ryan

Journal Article GENERALIZED PUSTULAR PSORIASIS: A CLINICAL AND EPIDEMIOLOGICAI. STUDY OF 104 CASES Get access HARVEY BAKER, HARVEY BAKER Institute of Dermatology, St. John's Hospital, London Search for other works … Journal Article GENERALIZED PUSTULAR PSORIASIS: A CLINICAL AND EPIDEMIOLOGICAI. STUDY OF 104 CASES Get access HARVEY BAKER, HARVEY BAKER Institute of Dermatology, St. John's Hospital, London Search for other works by this author on: Oxford Academic Google Scholar TERENCE J. RYAN TERENCE J. RYAN Institute of Dermatology, St. John's Hospital, London Search for other works by this author on: Oxford Academic Google Scholar British Journal of Dermatology, Volume 80, Issue 12, 1 December 1968, Pages 771–793, https://doi.org/10.1111/j.1365-2133.1968.tb11947.x Published: 01 December 1968 Article history Accepted: 17 May 1968 Published: 01 December 1968

SEROLOGICAL EVIDENCE OF AN ASSOCIATION OF A NOVEL CHLAMYDIA, TWAR, WITH CHRONIC CORONARY HEART DISEASE AND ACUTE MYOCARDIAL INFARCTION

1988-10-01

Pekka Saikku , Kari Mattila , Markku S. Nieminen +5 more

Consensus statement on definitions of disease, end points, and therapeutic response for pemphigus

2008-03-15

Dédée F. Murrell , Sarah Dick , A. R. Ahmed +7 more

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Distinct functions for integrins alpha 3 beta 1 in focal adhesions and alpha 6 beta 4/bullous pemphigoid antigen in a new stable anchoring contact (SAC) of keratinocytes: relation to hemidesmosomes.

1990-12-01

W G Carter , Pritinder Kaur , Susana G. Gil +2 more

Basal cells of stratified epidermis are anchored to the basement membrane zone (BMZ) of skin via hemidesmosomes. We previously identified integrin alpha 3 beta 1, in focal adhesions (FAs), of … Basal cells of stratified epidermis are anchored to the basement membrane zone (BMZ) of skin via hemidesmosomes. We previously identified integrin alpha 3 beta 1, in focal adhesions (FAs), of cultured human keratinocytes (HFKs) as a mediator of HFK adhesion to secreted BMZ-like extracellular matrix (ECM; Carter, W.G., E.A. Wayner, T.S. Bouchard, and P. Kaur. 1990. J. Cell Biol. 110: 1387-1404). Here, we have examined the relation of integrins alpha 6 beta 4 and alpha 3 beta 1, to bullous pemphigoid antigen (BPA), a component of hemidesmosomes. We conclude that alpha 6 beta 4 in HFKs localizes in a new stable anchoring contact (SAC) that cooperates with alpha 3 beta 1-FAs to mediate adhesion to ECM, based on the following. (a) Comparison of secreted ECM, with exogenous laminin, fibronectin and collagen identified ECM as the preferred ligand for HFK adhesion and spreading and for formation of both alpha 6 beta 4-SACs and alpha 3 beta 1-FAs. (b) Inhibition of HFK adhesion with combined anti-alpha 3 beta 1 (P1B5) and anti-alpha 6 beta 4 (GoH3) antibodies indicated that both receptors were functional in adhesion to ECM while alpha 3 beta 1 played a dominant role in spreading. (c) alpha 6 beta 4 colocalized with BPA in SACs that were proximal to but excluded from FAs. Both alpha 6 beta 4-SACs and alpha 3 beta 1-FAs were in contact with the adhesion surface as indicated by antibody exclusion and interference reflection microscopy. (d) In contrast to alpha 3 beta 1-FAs, alpha 6 beta 4-SACs were present only in nonmotile cells, not associated with stress fibers, and were relatively stable to detergents and urea, suggesting a nonmotile, or anchoring function for SACs and motility functions for alpha 3 beta 1-FAs. (e) alpha 6 beta 4 formed a detergent-insoluble complex with exogenous ECM in an affinity isolation procedure, confirming the ability of an unidentified ECM ligand to interact with alpha 6 beta 4. (f) We suggest that alpha 6 beta 4/BPA-SACs in culture restrict migration of HFKs on ECM while alpha 3 beta 1-FAs form dynamic adhesions in spreading and migrating cells. alpha 6 beta 4/BPA-SACs in culture bear functional and compositional similarities to hemidesmosomes in skin.

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Pemphigus, Bullous Impetigo, and the Staphylococcal Scalded-Skin Syndrome

2006-10-25

John R. Stanley , Masayuki Amagai

This review discusses the autoantibodies in pemphigus vulgaris and pemphigus foliaceus and the clinical and pathological similarities among these forms of pemphigus, bullous impetigo, and the staphylococcal scalded-skin syndrome. This review discusses the autoantibodies in pemphigus vulgaris and pemphigus foliaceus and the clinical and pathological similarities among these forms of pemphigus, bullous impetigo, and the staphylococcal scalded-skin syndrome.

Targeted Disruption of the Pemphigus Vulgaris Antigen (Desmoglein 3) Gene in Mice Causes Loss of Keratinocyte Cell Adhesion with a Phenotype Similar to Pemphigus Vulgaris

1997-06-02

Peter J. Koch , Mỹ G. Mahoney , Hiroyasu Ishikawa +6 more

In patients with pemphigus vulgaris (PV), autoantibodies against desmoglein 3 (Dsg3) cause loss of cell–cell adhesion of keratinocytes in the basal and immediate suprabasal layers of stratified squamous epithelia. The … In patients with pemphigus vulgaris (PV), autoantibodies against desmoglein 3 (Dsg3) cause loss of cell–cell adhesion of keratinocytes in the basal and immediate suprabasal layers of stratified squamous epithelia. The pathology, at least partially, may depend on protease release from keratinocytes, but might also result from antibodies interfering with an adhesion function of Dsg3. However, a direct role of desmogleins in cell adhesion has not been shown. To test whether Dsg3 mediates adhesion, we genetically engineered mice with a targeted disruption of the DSG3 gene. DSG3 −/− mice had no DSG3 mRNA by RNase protection assay and no Dsg3 protein by immunofluorescence (IF) and immunoblots. These mice were normal at birth, but by 8–10 d weighed less than DSG3 +/− or +/+ littermates, and at around day 18 were grossly runted. We speculated that oral lesions (typical in PV patients) might be inhibiting food intake, causing this runting. Indeed, oropharyngeal biopsies showed erosions with histology typical of PV, including suprabasilar acantholysis and “tombstoning” of basal cells. EM showed separation of desmosomes. Traumatized skin also had crusting and suprabasilar acantholysis. Runted mice showed hair loss at weaning. The runting and hair loss phenotype of DSG3 −/− mice is identical to that of a previously reported mouse mutant, balding (bal). Breeding indicated that bal is coallelic with the targeted mutation. We also showed that bal mice lack Dsg3 by IF, have typical PV oral lesions, and have a DSG3 gene mutation. These results demonstrate the critical importance of Dsg3 for adhesion in deep stratified squamous epithelia and suggest that pemphigus autoantibodies might interfere directly with such a function.

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The specific dermatoses of pregnancy revisited and reclassified: Results of a retrospective two-center study on 505 pregnant patients

2006-02-20

Christina M. Ambros‐Rudolph , Robert R. Müllegger , Samantha A. Vaughan-Jones +2 more

Identification of the Skin Basement-Membrane Autoantigen in Epidermolysis Bullosa Acquisita

1984-04-19

David T. Woodley , Robert A. Briggaman , Edward J. O’Keefe +3 more

Epidermolysis bullosa acquisita is an acquired chronic blistering disease of the skin, in which separation of the skin occurs in the basement-membrane zone between the epidermis and the dermis. There … Epidermolysis bullosa acquisita is an acquired chronic blistering disease of the skin, in which separation of the skin occurs in the basement-membrane zone between the epidermis and the dermis. There is evidence that blistering is initiated by an immune process. Using serum samples from nine patients as a source of antibodies, we have identified a major protein of the basement membrane of human skin that serves as the antigen (or target) for autoantibodies in this disorder. This previously unrecognized protein, which consists of two components of 290,000 and 145,000 daltons, is distinct from other known components of the basement membrane. These studies provide evidence that epidermolysis bullosa acquisita is a specific disease that is different from other primary bullous diseases, such as bullous pemphigoid and pemphigus vulgaris, and suggest that the basement-membrane component that has been identified may have a role in normal epidermal–dermal adherence. (N Engl J Med 1984; 310:1007–13.)

Treatment of Pemphigus Vulgaris with Rituximab and Intravenous Immune Globulin

2008-01-01

J.A. Stockman

Demonstration of Skin Antibodies in Sera of Pemphigus Vulgaris Patients by Indirect Immunofluorescent Staining.

1964-11-01

Ernst H. Beutner , R E Jordon

Summary1. Eight of 13 sera from patients with pemphigus vulgaris were found to contain antibodies to a substance at the surface of the cells of stratified squamous epithelium, particularly in … Summary1. Eight of 13 sera from patients with pemphigus vulgaris were found to contain antibodies to a substance at the surface of the cells of stratified squamous epithelium, particularly in the stratum spinosum, as demonstrated by indirect immunofluores-cent (I.I.F.) staining. The reactions of 4 of these 8 sera were deemed to be weakly positive or doubtful while the remaining 4 sera yielded titers of 1:30 to 1:120 by I.I.F. staining. The reactive antigen was found only in stratified squamous epithelium. Other types of epithelial tissue studied to date do not appear to contain the antigen. None of the 88 sera of normal individuals, patients with other skin diseases, or patients with other immunologic disturbances that have been tested to date appear to contain such antibody activity. However, 2 of the sera of patients with other chronic bullous skin diseases appeared to contain an antibody to the basement membrane of the skin and other stratified squamous epithelia. 2. A skin biopsy of one of the antibody producing patients with pemphigus vulgaris revealed the presence of bound gamma globulin on the surface of the epithelial cells in an apparently normal portion of the skin adjacent to a vesicle. This localization was identical to that obtained by I.I.F. staining with the patient's serum.The authors gratefully acknowledge instruction and suggestions in the field of immunology by Dr. Ernest Witebsky, suggestions of Dr. James Jordon which stimulated the initiation of this study as well as his supply of the first 3 sera, the supply of one serum and skin biopsies by Dr. Joseph Aquilina, and the critique of the manuscript and supply of some additional sera by Dr. Walter Lever.

[Intercapillary deposits of IgA-IgG].

1968-09-01

J. Berger , N Hinglais

Liquor amnii analysis in the management of the pregnancy complicated by rhesus sensitization

1961-12-01

A. W. Liley

First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomised trial

2017-03-23

P. Joly , Maud Maho‐Vaillant , C. Prost‐Squarcioni +7 more

Mechanisms of Autoantibody-Induced Pathology

2017-05-31

Ralf J. Ludwig , Karen Vanhoorelbeke , Frank Leypoldt +7 more

Autoantibodies are frequently observed in healthy individuals. In a minority of these individuals, they lead to manifestation of autoimmune diseases, such as rheumatoid arthritis or Graves' disease. Overall, more than … Autoantibodies are frequently observed in healthy individuals. In a minority of these individuals, they lead to manifestation of autoimmune diseases, such as rheumatoid arthritis or Graves' disease. Overall, more than 2.5% of the population is affected by autoantibody-driven autoimmune disease. Pathways leading to autoantibody-induced pathology greatly differ among different diseases, and autoantibodies directed against the same antigen, depending on the targeted epitope, can have diverse effects. To foster knowledge in autoantibody-induced pathology and to encourage development of urgently needed novel therapeutic strategies we here categorized autoantibodies according to their effects. According to our algorithm, autoantibodies can be classified into the following categories: (1) Mimic receptor stimulation, (2) blocking of neural transmission, (3) induction of altered signaling, triggering uncontrolled (4) microthrombosis, (5) cell lysis, (6) neutrophil activation and (7) induction of inflammation. These mechanisms in relation to disease, as well as principles of autoantibody generation and detection are reviewed herein.

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Histologic Diagnosis of Inflammatory Skin Diseases.

1979-06-01

Angio-immunoblastic lymphadenopathy with dysproteinemia

1974-01-01

Glauco Frizzera

Pemphigus

2019-09-01

Enno Schmidt , Michael Kasperkiewicz , P. Joly

Pemphigus

2017-05-10

Michael Kasperkiewicz , Christoph T. Ellebrecht , Hayato Takahashi +4 more

Pemphigoid diseases

2012-12-11

Enno Schmidt , Detlef Zillikens

PEMPHIGUS

1953-02-01

Walter F. Lever

*From the Department of Dermatology, Harvard Medical School, and the Dermatologic Service, Massachusetts General Hospital, Boston, Mass. *From the Department of Dermatology, Harvard Medical School, and the Dermatologic Service, Massachusetts General Hospital, Boston, Mass.

Paraneoplastic pemphigus

2007-08-01

Xuejun Zhu , Bingxin ZHANG

Paraneoplastic pemphigus (PNP) is a life-threatening autoimmune blistering skin disease. Clinically, it is characterized by severe mucosal erosions and various cutaneous lesions associated with lymphoproliferative neoplasmas. Suprabasal acantholysis and clefts … Paraneoplastic pemphigus (PNP) is a life-threatening autoimmune blistering skin disease. Clinically, it is characterized by severe mucosal erosions and various cutaneous lesions associated with lymphoproliferative neoplasmas. Suprabasal acantholysis and clefts with scattered necrotic keratinocytes are the unique histopathological features. PNP patient sera recognize multiple antigens, which have been identified as the plakin protein family that includes desmoplakin, bullous pemphigoid antigen I (BPAG1), envoplakin and periplakin, and desmogleins 1 and 3. Castleman's tumor, non-Hodgkin's lymphoma, thymoma, follicular dendritic cell sarcoma and chronic lymphocytic leukemia are the commonly associated neoplasmas in PNP. We have also demonstrated that the autoantibodies reacting to epidermal proteins are directly produced by the cells in the associated tumors. Bronchiolitis obliterans is frequently found in PNP and may cause respiratory failure and death. In our experience, the early detection and removal of the tumor and i.v. administration of immunoglobulin are critical for the treatment of PNP.

Dermatological formulations: Percutaneous absorption

1984-01-01

Brian Barry

Clinical Etiology and Histopathologic Correlation of Exfoliative Erythroderma at the Kenyatta National Hospital

2025-06-25

Karen Waithera Wainaina , Priscilla Angwenyi , Beatrice Wangari Ndege | East African Journal of Health and Science

Background: Exfoliative erythroderma is a dermatologic emergency characterised by diffuse skin redness and scaling involving at least 70% body surface area. It is a clinical presentation that is usually indicative … Background: Exfoliative erythroderma is a dermatologic emergency characterised by diffuse skin redness and scaling involving at least 70% body surface area. It is a clinical presentation that is usually indicative of an underlying primary process. Once a clinical diagnosis of exfoliative erythroderma is made, prompt supportive measures should be instituted while seeking to identify the underlying cause of this presentation. The correlation between clinical and histopathological diagnoses has not been determined for the Kenyan population. Purpose of the study: To assess the frequency of causes of exfoliative erythroderma and their histopathologic correlation at Kenyatta National Hospital. Methodology: This was an ambispective study conducted in Kenyatta National Hospital wards and clinics. All adult patients with exfoliative erythroderma who meet the study criteria will be included. A total sample size of 94 patients was included in the study. Descriptive analysis was done where demographic, clinical and histopathological factors were summarised using mean and standard deviation as well as median and interquartile range. A sensitivity analysis was performed to correlate clinical findings and histopathological findings. Results: The median age was 45 (interquartile range: 30 – 60 years, and 53% of them were male. Clinical causes of EE revealed that most of the patients with EE were due to psoriasis (38.3%), followed by malignancies (21.3%) and eczema (18.1%). Clinical findings revealed that the common causes of EE included dermatoses (57.45%), with psoriasis (24.2%) followed by eczema (18.1%). Malignancies (21.3%) were the second most common group and the commonest systemic disease, followed by drug reactions (12.7%). HIV was present in 7.5% of the cases. The findings showed that 63% (n =57) of the patients had a biopsy done, although the frequency was lower in the retrospective arm, 57% (n =42), compared to the prospective arm of the study, 88% (n =15). The findings from the histopathological findings revealed that 39.2% were malignancies, followed by psoriasis 25.5%) and immunobullous disease (17.7%). The sensitivity analysis revealed that immunobullous (100%) and malignancy (92.3%) had a high level of sensitivity as well as specificity, which was 97.7% and 92.6%, respectively, although eczema and psoriasis were least correlated with histopathological findings. Conclusion: Clinical findings were better correlated with malignancy and immunobullous findings with high sensitivity and specificity, although the other histopathology findings, such as psoriasis and eczema, were poorly correlated. Thus, there is a continued need to adopt a biopsy‐first protocol for all new presentations of EE to maximise diagnostic yield.

Breaking the blisters: a comprehensive guide to treating bullous pemphigoid

2025-06-24

Amber Subhan , Lolla Siddhartha , Mudimala Rekha Goud +1 more | International Journal of Basic & Clinical Pharmacology

In adapting the treatment plan, the severity of the disease in correlation with the patient should be put onto consideration. Recent RCTs demonstrated adjuvant treatment with doxycycline, dapsone, and immunosuppressants … In adapting the treatment plan, the severity of the disease in correlation with the patient should be put onto consideration. Recent RCTs demonstrated adjuvant treatment with doxycycline, dapsone, and immunosuppressants for the treatment of bullous pemphigoid is of advantage and safe in terms of diminished total steroid dose and mortality. The British association of dermatologists has produced dermatologists’ guidelines. These provide evidence-based treatment recommendations with respect to candidness, and offer a summary on, among others, epidemiological features, diagnosis, and research. The evidence on which the guidelines rest was retrieved from Medline, Embase, the Cochrane Library, literature searches, and the authors' 10 years of experience treating patients with bullous pemphigoid in general and special clinics. However, it should be noted that findings from the literature should be interpreted with great care as only six randomized controlled trials with small patient groups are available.

Clinical, Serologic, and Histopathologic Features of Patients With Pemphigus With Either Positive or Negative <scp>IgG4</scp> Intercellular Deposition by Direct Immunofluorescence (<scp>DIF</scp>): A Retrospective Case–Control Study of 55 <scp>DIF</scp> Biopsy Specimens

2025-06-24

Clint Christian T. Garbanzos , Austin Todd , Heather D Hardway +1 more | Journal of Cutaneous Pathology

ABSTRACT Background Intercellular IgG4 deposition is variably present on DIF in patients with pemphigus. Whether this feature has clinical, serologic, or histopathologic correlates was unknown. Methods We identified 34 patients … ABSTRACT Background Intercellular IgG4 deposition is variably present on DIF in patients with pemphigus. Whether this feature has clinical, serologic, or histopathologic correlates was unknown. Methods We identified 34 patients with pemphigus who had 55 DIF specimens reported to show intercellular IgG, IgG4, and/or C3 deposition (8/22/2017–11/30/2023). Patients and biopsies were stratified by intercellular IgG4 status. Clinical and serologic data were extracted from electronic records, and corresponding biopsy slides were reviewed for histopathologic findings. Results Among the 34 patients with pemphigus, patients with positive IgG4 were significantly more likely to have detectable serum anti‐desmoglein 1/3 antibodies by ELISA ( p = 0.014 and p = 0.030, respectively). Paraneoplastic pemphigus (PNP) was more frequent in the IgG4‐negative group ( p = 0.037), particularly among patients with ocular involvement. Of 55 DIF biopsy specimens meeting inclusion criteria, 52 (94.5%) had intercellular IgG deposition and 42 (76.4%) had intercellular IgG4 on DIF. Compared to IgG4‐negative specimens, IgG4‐positive specimens were significantly more likely to represent a vesiculobullous lesion ( p = 0.024), to be derived from the trunk ( p = 0.047), to show histologic acantholysis ( p = 0.019) and to lack lichenoid inflammation ( p = 0.0004). Conclusion For pemphigus, DIF IgG4 status correlated with ocular involvement, the clinical morphology selected for biopsy, the likelihood of detecting circulating desmoglein antibodies, the presence of specific histopathologic features such as acantholysis and lichenoid inflammation, and the likelihood of having a final diagnosis of PNP. Further studies are needed to determine whether the presence of tissue‐bound intercellular IgG4 antibodies correlates with particular disease variants or lesion‐specific characteristics.

The Role of Microbiota in the Pathogenesis of Bullous Pemphigoid and Pemphigus Vulgaris: Evidence, Controversies, and Perspectives

2025-06-24

Francesca Gorini , Alessio Coi , Michele Santoro +7 more | International Journal of Molecular Sciences

Bullous pemphigoid (BP) and pemphigus vulgaris (PV) represent the most prevalent conditions among autoimmune bullous skin diseases, considered a major cause of severe morbidity and, in certain cases, mortality. The … Bullous pemphigoid (BP) and pemphigus vulgaris (PV) represent the most prevalent conditions among autoimmune bullous skin diseases, considered a major cause of severe morbidity and, in certain cases, mortality. The hallmark of the two diseases is the presence of autoantibodies directed against proteins located in the basement membrane of the skin, which determines the formation of blisters. In recent years, interest in the role of microbiota in relation to health-disease status has progressively increased. In particular, based on the gut–skin axis, accumulating evidence has emerged on the potential association between the composition and diversity of microbial communities in the gut, skin, and even in the oral cavity and the risk of developing BP and PV. Dysbiosis, characterized by a generally higher relative abundance of Firmicutes and a depletion of probiotics/beneficial species, might contribute to the pathogenesis of both diseases. Despite the still limited number of studies and the need for further large-scale multicenter studies, the knowledge gathered so far is suggestive of a novel modifiable risk factor representing a potential target for adjuvant treatments of these disabling and life-threatening conditions.

The clinical phenotypes and therapeutic strategies for stiff skin syndrome: a case series with literature review

2025-06-23

Caihui Zhang , Sihao Gao , Zhixing Sun +2 more | Orphanet Journal of Rare Diseases

Stiff skin syndrome (SSS) is a rare, non-inflammatory skin disease with a pronounced restriction in joint mobility. In this study, we aim to report Chinese pediatric patients with SSS in … Stiff skin syndrome (SSS) is a rare, non-inflammatory skin disease with a pronounced restriction in joint mobility. In this study, we aim to report Chinese pediatric patients with SSS in our center and summarize the clinical features of the disease through literature review. A retrospective study was conducted on 16 pediatric patients diagnosed with SSS at Peking Union Medical College Hospital between January 2014 and January 2024, based on clinical manifestations, laboratory tests, and skin biopsy findings. Among these cases, two were classified as widespread SSS, and 14 as segmental SSS. Additionally, a review of relevant literature published between January 2000 and January 2024 involving 138 cases of pediatric SSS was also conducted. The clinical characteristics, treatment, and prognosis of these 154 patients were summarized. The age of onset in patients was 2.0(0.5, 4.8) years, with an average age at diagnosis being 9.0(5.0, 13.0) years. Thigh skin sclerosis (81, 52.6%) was the most common manifestation observed in these patients. Joint restriction was present in 55(35.7%) patients. Patients with joint contractures had longer diagnostic delays compared with those without joint contractures. Patients were primarily treated with physical therapy, while some patients received medications such as mycophenolate mofetil (MMF), losartan, and secukinumab. However, the prognosis varied. The diagnosis of SSS should involve a thorough investigation of family history, detailed physical examination, comprehensive pathological assessment, genetic testing when applicable, and careful exclusion of other scleroderma-like diseases. Currently, there is limited evidence supporting the use of systemic treatment options targeting the transforming growth factor-β or interleukin-17 pathways (such as MMF, losartan, and secukinumab) to slow disease progression. However, these treatments are not capable of reversing established skin lesions, and further investigations are imperative to assess their therapeutic efficacy in SSS.

Superficial Pustular Folliculitis of the Face and Neck—A Non‐Infectious Eruption Responding to Topical Steroids

2025-06-23

Hui‐Peng Huang , Chang‐Ming Huang , Chao‐Kai Hsu +2 more | JEADV Clinical Practice

ABSTRACT Background We have seen patients presenting with tiny, superficial follicular pustules on the face and neck that appeared distinct from common or well‐known facial pustular dermatoses, such as acne … ABSTRACT Background We have seen patients presenting with tiny, superficial follicular pustules on the face and neck that appeared distinct from common or well‐known facial pustular dermatoses, such as acne vulgaris, rosacea, demodicosis, and Ofuji's disease. Objectives We aimed to describe the clinicopathologic features, differential diagnosis, and treatment of this pustular eruption in southern Taiwan. Methods We retrospectively reviewed the medical records and clinical photos of cases presenting with tiny, superficial follicular pustules on the face and/or neck during July 2017–March 2022. Cases of rosacea, acne vulgaris, demodicosis, and Ofuji's disease were excluded. Results A total of 27 patients (26 females and 1 male; mean age of 25.2 ± 4.3 years) were included for analysis. All patients presented with monomorphous, discrete, tiny, superficial pustules on the face and/or neck. The pustules varied from a few to hundreds in number. The eruptions were distributed on the face in 12 (44.4%) patients, the face and neck in 14 (51.8%), and the neck in one (3.7%), and was itchy in 44.4%. The pustules lasted 1 day to 1 month, mostly within 1 week, before treatment, and was recurrent in 81% of cases with 2–20 episodes individually. Twenty‐four of the 25 (96%) patients responded well to steroids with complete clearance of pustules. Skin biopsy of pustules performed in three cases showed infundibular pustules filled with neutrophils and a perivascular lymphohistiocytic infiltrate in the dermis. Gram staining revealed negative finding. Bacterial cultures performed in two patients revealed Cutibacterium acnes . Conclusions Based on our observation, we would like to propose the term ‘superficial pustular folliculitis of the face and neck’ (SPFFN) for this type of eruption. It is important to be familiar with this particular type of follicular pustulosis, especially when dealing with young females as the pustules respond well to low‐potency topical steroids.

Immunotherapy for pemphigus and bullous pemphigoid

2025-06-23

Chuqiao Xu , Yi Shen , Qijun Wang +2 more | Current Opinion in Immunology

A Clinico-Epidemiological Study of Various Autoimmune Vesiculobullous Disorders in a Tertiary Care Center

2025-06-22

Vunnava Sri Koulini , Dilipchandra Chintada , Kirankanth Vudayana +1 more | Cureus

What Are The Most Effective First-Line Pharmacological Treatments For Managing Bullous Pemphigoid In Elderly Patients Over 65 Years Old? : A Systematic Review

2025-06-22

Aulia Paramita , I Dewa Ayu Supriyantini , Ni Putu Devi Gema Partamayani +1 more | The International Journal of Medical Science and Health Research

Introduction: Bullous pemphigoid is an autoimmune blistering disease primarily affecting older adults. Managing this condition in patients over 65 is challenging, requiring a balance between controlling severe symptoms and minimizing … Introduction: Bullous pemphigoid is an autoimmune blistering disease primarily affecting older adults. Managing this condition in patients over 65 is challenging, requiring a balance between controlling severe symptoms and minimizing treatment-related risks. This review synthesizes evidence on the most effective and safe first-line pharmacological treatments for this vulnerable population. Methods: A systematic review was conducted following PRISMA guidelines. We searched PubMed, Semantic Scholar, Springer, and Google Scholar for randomized controlled trials, systematic reviews, and prospective cohort studies investigating first-line pharmacological treatments for bullous pemphigoid in patients over 65. Results: Systemic corticosteroids like prednisolone offer rapid disease control (91% achieving ≤3 blisters at 6 weeks) but are associated with a high rate of severe adverse events (36% at 1 year). Doxycycline was found to be non-inferior for short-term control (74% achieving ≤3 blisters at 6 weeks) with a significantly better safety profile, halving the rate of severe adverse events to 18%. High-potency topical corticosteroids were effective and well-tolerated for moderate disease. Discussion: The evidence supports a risk-stratified approach. While effective, the high toxicity of systemic corticosteroids makes them less ideal as a universal first-line choice. Doxycycline and high-potency topical corticosteroids represent a safer initial strategy, balancing good efficacy with significantly lower risk. Novel biologics, particularly dupilumab, are poised to become a new standard of care, offering high efficacy without the severe risks of broad immunosuppression, pending results from ongoing randomized controlled trials. Conclusion: For elderly patients with bullous pemphigoid, doxycycline and high-potency topical corticosteroids are the preferred first-line treatments due to their favorable balance of efficacy and safety. Systemic corticosteroids should be reserved for specific cases where rapid control is essential and risks are manageable. Novel therapies like dupilumab show great promise and may soon redefine the standard of care, pending final evidence from ongoing trials.

Ocrelizumab

2025-06-21

| Reactions Weekly

Assessment of Efficacy of Adjuvant Topical Rituximab Encapsulated in Nanoparticle Gel in Oral Pemphigus Vulgaris: A Randomized, Double-Blind, Placebo-Controlled, Pilot Study

2025-06-20

Pratik Mohta , Sujay Khandpur , Amit Kumar Dinda +4 more | Indian Dermatology Online Journal

Abstract Background: Oral pemphigus vulgaris (PV) presents with persistent, painful, nonhealing oral erosions, with a slower treatment response compared to cutaneous lesions. Patients and Methods: To assess the efficacy of … Abstract Background: Oral pemphigus vulgaris (PV) presents with persistent, painful, nonhealing oral erosions, with a slower treatment response compared to cutaneous lesions. Patients and Methods: To assess the efficacy of adjuvant topical rituximab encapsulated in nanoparticle gel in oral PV. Of 31 oral PV patients recruited, 16 were randomized to rituximab incorporated in calcium alginate nanoparticles (study group) and 15 to calcium alginate nanoparticle gel only (placebo group), applied twice daily on oral erosions for 12 weeks. Both groups, in addition, received oral prednisolone (tapering doses) and azathioprine. Results: In the topical rituximab group, 75% of patients and in the placebo group, 86.7% of patients achieved remission ( P = 0.65) over 12 weeks of therapy. The median time to remission in the topical rituximab group was 4 weeks, and in the placebo group, it was 8 weeks ( P = 0.53). Median oral pemphigus scores showed an earlier reduction in the rituximab group ( P = 0.52). The median circulating CD20 count was reduced in the topical rituximab group from 100 to 74/mm 3 at week 8. In the placebo group, the value increased from 89 to 118/mm 3 (intergroup P = 0.29). A significantly greater number of cases in the topical rituximab group had a negative mucosal CD20 count ( P = 0.001). There was a comparable fall in desmoglein 3 titers in both groups ( P = 0.58). The mean cumulative prednisolone dose was 3567 mg in the topical rituximab group and 3869 mg in the placebo group ( P = 0.125). No side effects of rituximab were observed. Limitations: A small sample size, and need of pharmacokinetic studies in animal models to assess absorption of rituximab in nanogel formulation. Conclusion: With topical rituximab incorporated in nanogel, there was a trend toward earlier remission, lower total cumulative oral prednisolone dose, earlier improvement in oral pemphigus score, and greater reduction in CD20 count.

Bullous Pemphigoid: Clinical Aspects and Treatments

2025-06-20

Ryan S Q Geng , R. Gary Sibbald | Advances in Skin & Wound Care

ABSTRACT Bullous pemphigoid (BP) is the most common subepidermal blistering disease. It is typically observed in the older adult population and rarely in children. Classic BP is characterized by tense … ABSTRACT Bullous pemphigoid (BP) is the most common subepidermal blistering disease. It is typically observed in the older adult population and rarely in children. Classic BP is characterized by tense blisters filled with clear or hemorrhagic fluid on normal skin or erythematous base with predilection for flexor surfaces. The criterion standard for diagnosing BP is with direct immunofluorescence for detection of linear deposition of immunoglobulin G or C3 at the dermoepidermal junction and/or indirect immunofluorescence for immunoglobulin G at the basement membrane zone. Treatments with efficacy in treating BP rely on anti-inflammatory or immunosuppressive properties, with corticosteroids being the primary choice of treatment. This review focuses on the clinical presentation, epidemiology, risk factors, and treatment options for BP. GENERAL PURPOSE: To review the risk factors and clinical features of bullous pemphigoid (BP) and discuss available treatment options. TARGET AUDIENCE This continuing education activity is intended for physicians, physician assistants, nurse practitioners, and registered nurses with an interest in skin and wound care. LEARNING OBJECTIVES/OUTCOMES: After participating in this educational activity, the participant will: 1. Summarize the clinical features and manifestations associated with BP. 2. Identify evidence-based methods to diagnose BP. 3. Explain evidence-based pharmacologic management strategies for the effective treatment of BP.

Bullous Pemphigoid: Clinical Aspects and Treatments

2025-06-20

| Advances in Skin & Wound Care

Autoimmune blistering skin disease classification: classical machine learning, deep learning, and hybrid approaches

2025-06-20

Manbir Singh , Maninder Singh , Dipankar De +4 more | Neural Computing and Applications

Diagnóstico de penfigoide anti-p200 sin inmunoblot: utilidad de la inmunohistoquímica para colágeno IV

2025-06-20

Marta Cebolla-Verdugo , David Moyano-Bueno , Ricardo Ruíz‐Villaverde | Medicina Clínica

Cost implications of European guidelines for whole‐body clobetasol for pemphigoid in the United States

2025-06-20

Sabrina F. Schundler , M Mihăilescu , Kyle T. Amber | Journal of the European Academy of Dermatology and Venereology

Benign familial pemphigus

2025-06-20

Adolfo Contreras , Elena Soler , Cristina Albanell Fernández | Medicina Clínica

Clinical Characteristics, Management, and Outcomes of Pemphigoid Nodularis: A Systematic Review

2025-06-19

Darshana Seeburruth , Ragav Chona , Nicholas Hua +3 more | Journal of Cutaneous Medicine and Surgery

Oral pemphigus vulgaris with laryngeal involvement

2025-06-19

Linger Sim | Visual Journal of Emergency Medicine

Can we cure bullous skin diseases?

2025-06-19

Siyao Liu , Qianjin Lu | Current Opinion in Immunology

Optimising Vaccination Status in a Belgian Dermatological Immune‐Mediated Inflammatory Disease Population: An Education‐Based Pro‐Active Patient‐Centred Approach

2025-06-19

Femke Lieten , Tine Vanhoutvin , Dorien Hunin +6 more | JEADV Clinical Practice

ABSTRACT Background Patients with dermatological immune mediated inflammatory diseases (IMIDs) are increasingly treated with immunosuppressive and ‐modulating drugs. Some of these drugs increase the risk of acquiring infections and more … ABSTRACT Background Patients with dermatological immune mediated inflammatory diseases (IMIDs) are increasingly treated with immunosuppressive and ‐modulating drugs. Some of these drugs increase the risk of acquiring infections and more complications can arise during an infection while treated with these agents. Accurate vaccination can prevent these infections and associated complications. Objectives To map the vaccination status of dermatological IMID patients receiving immunosuppressive systemic treatment and evaluate the impact of targeted educational interventions on improving it. We also addressed the awareness on the subject among healthcare providers (HCPs). Methods In this monocentric prospective study, we mapped the influenza, pneumococcal, hepatitis B and COVID‐19 vaccination status of patients with dermatological IMIDs receiving immunosuppressive treatment (baseline) via the national vaccination register (Vaccinnet). We educated patients and HCPs (general practitioners and pharmacists) on the importance and need of vaccination. Two years later their vaccination status was reassessed (follow‐up). Results From March 2022 until December 2022, we identified 314 patients treated with immunosuppressive drugs for dermatological IMIDs at UZ Leuven. At baseline, vaccination rates were 56.1% for influenza, 50.2% for pneumococcal disease, and 46.0% for hepatitis B. Older individuals (≥ 40 years old) had significantly higher vaccination rates for influenza ( p = 0.002), pneumococcal ( p = 0.002), and COVID‐19 vaccines ( p = 0.004), but lower rates for hepatitis B vaccination ( p < 0.001) compared to younger patients. At follow‐up, influenza vaccination rates remained stable (57.0%, p = 0.579), while pneumococcal, hepatitis B and COVID‐19 vaccination rates significantly increased to 60.6% ( p < 0.001), 50.0% ( p = 0.002), and 80% ( p < 0.001), respectively. Notably, a subset of 55 patients initially registered as vaccinated for influenza at baseline were later categorised as unvaccinated, largely due to missing registrations in Vaccinnet. Conclusions Thorough education of patients with dermatological IMIDs and HCPs can result in an additional increase of vaccination rates in times of vaccination fatigue. Meticulous registration of administered vaccinations by HCPs is imperative.

Exploring the role of ferroptosis in pemphigus: identification of diagnostic markers and regulatory mechanisms

2025-06-19

Jing Mao , Jianping Lan , Zheyu Zhuang +7 more | Frontiers in Medicine

Background Pemphigus is an autoimmune blistering disorder characterized by the loss of cell adhesion in the epidermis. Recent studies have suggested a potential link between ferroptosis, a form of regulated … Background Pemphigus is an autoimmune blistering disorder characterized by the loss of cell adhesion in the epidermis. Recent studies have suggested a potential link between ferroptosis, a form of regulated cell death dependent on iron, and various diseases. However, the role of ferroptosis-related genes in pemphigus remains largely unexplored. This study aims to investigate the expression patterns and potential biological functions of ferroptosis-related genes in pemphigus, as well as their regulatory mechanisms. Methods To achieve this, skin samples from five pemphigus patients and five healthy controls were collected from Fujian Medical University Union Hospital. Additionally, we processed the GSE53873 microarray dataset, which includes 19 pemphigus samples and 5 controls. Differentially expressed genes (DEGs) were identified using the limma R package, followed by Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) enrichment analyses. Weighted Gene Co-expression Network Analysis (WGCNA) was employed to identify co-expressed gene modules related to pemphigus. Machine learning algorithms such as Least Absolute Shrinkage and Selection Operator (LASSO), Random Forest (RF), and eXtreme Gradient Boosting (XGBoost) were used to select key ferroptosis-related genes. Immune cell infiltration was assessed using CIBERSORT and single-sample Gene Set Enrichment Analysis (ssGSEA). Finally, experimental validation was conducted through real-time quantitative PCR, transmission electron microscopy, and drug prediction. Results Our results identified 1,840 DEGs in pemphigus patients compared to controls, with significant enrichment in pathways such as PI3K-Akt signaling and fatty acid metabolism. Eleven co-expression modules were identified via WGCNA, with the module highlighted in lightcyan color showing the strongest correlation with pemphigus. Machine learning highlighted ACSL4, SAT2, and XBP1 as potential hub genes with high diagnostic value. Immune analysis revealed increased proportions of activated CD8 + T cells and natural killer cells in pemphigus patients. Experimental validation confirmed the presence of ferroptosis morphological features in patient samples. Conclusion In conclusion, this study elucidates the involvement of ferroptosis-related genes in pemphigus pathogenesis and identifies potential biomarkers for diagnosis. Further research is warranted to explore therapeutic strategies targeting these pathways.

Polylactic Acid Membranes, a Novel Adjunct Treatment for Bullous Impetigo

2025-06-19

Ana Lorena Novoa-Moreno , Mario Aurelio Martínez‐Jiménez , Arturo Ortiz-Álvarez +3 more | Infectious Disease Reports

Impetigo is a highly contagious bacterial skin infection characterized by blistering and erosions that can lead to significant discomfort and complications. The standard treatment includes topical or systemic antibiotics, but … Impetigo is a highly contagious bacterial skin infection characterized by blistering and erosions that can lead to significant discomfort and complications. The standard treatment includes topical or systemic antibiotics, but severe cases may require advanced wound management strategies. Polylactic acid (PLA)-based membranes have demonstrated effectiveness in enhancing wound healing, modulating inflammation, and reducing pain. Clinical case: We present three cases of bullous impetigo with extensive erosions, managed using PLA membranes as an adjunct to systemic antibiotics. A significant improvement was shown after 7 days of treatment of a single application, and complete resolution was achieved after 30 days. Notably, pain was resolved within 48-72 h, highlighting the analgesic and protective properties of the membrane. Conclusions: These findings suggest that PLA membranes provide a viable adjunct to antibiotic therapy in bullous impetigo, accelerating healing, reducing discomfort, and improving long-term skin outcomes. Given the increasing concern over antibiotic resistance and the limitations of standard wound care, bioresorbable synthetic membranes represent a promising alternative in dermatological wound management.

Angina bullosa hemorrhagica: A rare and benign oral mucosal disorder – A case report

2025-06-18

Diksha Shukla , Meenakshi Upadhyay , S. Ali Raza +1 more | Asian Journal of Oral Health and Allied Sciences

The oral cavity plays a crucial role in overall health, with certain oral lesions potentially indicating underlying conditions. Angina bullosa hemorrhagica (ABH) is a rare, benign oral mucosal disorder characterized … The oral cavity plays a crucial role in overall health, with certain oral lesions potentially indicating underlying conditions. Angina bullosa hemorrhagica (ABH) is a rare, benign oral mucosal disorder characterized by spontaneous blood-filled bullae unrelated to systemic diseases or bleeding disorders. This case report aims to describe a clinical presentation and management of a patient with recurrent ABH. A 17-year-old patient presented with a blood-filled lesion on the maxillary left buccal mucosa, persisting for one week, causing discomfort while chewing, with a history of a similar episode nine months back. No systemic or hematologic abnormalities were detected. Clinical examination revealed a solitary, tense, blood-filled bulla. Histopathology confirmed the diagnosis of ABH, ruling out other vascular or inflammatory lesions. There are no established guidelines for managing this condition. In the present case, an excisional biopsy was performed to distinguish it from other blood-containing bullae and alleviate discomfort. Follow-up was carried out at 15 days, 6 months, and 1 year. In conclusion, ABH is a rare but significant condition requiring accurate diagnosis to avoid diagnostic dilemmas and to prevent unnecessary treatments. Awareness of its clinical features helps clinicians differentiate it from other oral vesiculobullous disorders, ensuring appropriate patient management.

Intravenous immunoglobulin as an effective treatment for refractory pemphigus vegetans: A case report

2025-06-18

A. Ouled Moktar , Shuruq Alanazi , Sarah Anwar Almulla +1 more | Electronic Journal of General Medicine

Pemphigus vegetans (PVeg), which affects &lt; 2% of pemphigus cases, presents a therapeutic challenge when refractory to conventional treatments. Herein, we describe the case of a 58-year-old woman diagnosed with … Pemphigus vegetans (PVeg), which affects &lt; 2% of pemphigus cases, presents a therapeutic challenge when refractory to conventional treatments. Herein, we describe the case of a 58-year-old woman diagnosed with Hallopeau-type PVeg who demonstrated a complete response to intravenous immunoglobulin (IVIG) after failing multiple conventional therapies. The patient exhibited vegetative plaques at multiple sites, with notable nail involvement. Her disease remained poorly controlled despite a 7-year course of systemic corticosteroids, immunosuppressants, and rituximab. Two courses of IVIG (5% concentration, 2 g/kg) resulted in the complete resolution of the disease. This case highlights IVIG’s therapeutic potential in refractory PVeg and emphasizes the importance of recognizing atypical manifestations, including nail involvement. While this single case yielded promising results, future studies must validate the role of IVIG in the management of treatment-resistant PVeg.

Laryngeal Bullous Pemphigoid: A challenging presentation of a rare disease

2025-06-18

Hussein Smaily , Matthieu Crouslé , Edith Hanna | European Archives of Oto-Rhino-Laryngology

Generalized Pustular Psoriasis

2025-06-18

Su‐Chi Ku , Ying‐Yi Chiang | New England Journal of Medicine

Recent advances in the genetics and innate immune cells of bullous pemphigoid

2025-06-18

Xiaoli Yang , Panling Wei , Zaixing Wang | Frontiers in Immunology

Bullous pemphigoid (BP) is a common autoimmune subepidermal blistering disease that primarily affects elderly patients. The pathogenesis of BP is complex, involving genetic, immune, and environmental factors. Recent evidence suggests … Bullous pemphigoid (BP) is a common autoimmune subepidermal blistering disease that primarily affects elderly patients. The pathogenesis of BP is complex, involving genetic, immune, and environmental factors. Recent evidence suggests that multiple genomic regions, particularly within the human leukocyte antigen (HLA)-II region, influence susceptibility to BP. Genetically predisposed individuals may carry susceptibility alleles that modulate the immune system, leading to an elevated risk of developing BP when exposed to the appropriate environmental triggers. Here, the present review discusses the genetics of BP and the critical role of the innate immune system in BP pathogenesis, focusing on the composition of innate immune cells.

Rheumatologic and Immunologic Manifestations of Epidermolytic Hyperkeratosis: A Special Case Description and Literature Review

2025-06-18

| Annals of Case Reports

Pênfigo vulgar simulando lúpus cutâneo – relato de caso

2025-06-17

Beatriz Lacerda Bezerra , Milena Sonely Mendonça Bezerra Lima , Allyssandra Maria Lima Rodriguês Maia +2 more | Brazilian Journal of Health Review

Introdução: Os pênfigos são buloses crônicas de caráter autoimune e progressivo. A formação de bolhas intraepidérmicas decorre da acantólise induzida por autoimunidade. Os corticosteroides são administrados como terapia inicial. Objetivo: … Introdução: Os pênfigos são buloses crônicas de caráter autoimune e progressivo. A formação de bolhas intraepidérmicas decorre da acantólise induzida por autoimunidade. Os corticosteroides são administrados como terapia inicial. Objetivo: Analisar a evolução de quadro clínico de pênfigo vulgar em paciente em tratamento para lúpus eritematoso Métodos: Relato de caso, de natureza descritiva e retrospectiva, de uma paciente com Pênfigo vulgar simulando lúpus cutâneo, acompanhada em serviço ambulatorial. Relato de Caso: Paciente do sexo feminino, 38 anos, sob investigação de lúpus cutâneo, procura assistência médica referindo crepitações, dores articulares, além de evidenciar na ectoscopia placas hipercrômicas com centro crostoso e base eritematosa em região malar e de couro cabeludo. Foi referenciada para o serviço de dermatologia, após evolução com lesões bolhosas disseminadas. Em 2023, após múltiplas biópsias, teve o diagnóstico de pênfigo vulgar e foi iniciada a terapia com corticosteroide. Discussão: Os sinais e sintomas das doenças bolhosas por vezes se sobrepõem, a apresentação inicial do lúpus cutâneo cursa com o aparecimento de uma erupção localizada mais comum em região malar, a qual pode evoluir para um quadro sistêmico e bolhoso. Por outro lado, o pênfigo vulgar apresenta-se com a formação de bolhas intraepidérmicas, crescimento centrífugo e tamanhos variados. Ambas as patologias são de caráter autoimune e fazem diagnóstico diferencial importante possuindo terapias distintas. Conclusão: Constatou-se a sobreposição da clínica inicial de buloses, além da efetividade e eficácia da terapia medicamentosa quando o diagnóstico foi estabelecido.

Immunobullous Disorders

2025-06-17

Mai P. Hoang | Cambridge University Press eBooks

Impossible Teal

2025-06-17

Jacek Krywko | Scientific American

Influence of CD16 (FcγRIII) Expression on Peripheral Blood Mononuclear Cells on Response to Treatment with Rituximab in Pemphigus: A Cross-Sectional Study

2025-06-17

Ankur Sharma , Anshika Chauhan , Hitaishi Mehta +5 more | Indian Dermatology Online Journal

Abstract Background: Rituximab-mediated B-cell depletion in pemphigus patients is majorly caused by antibody-dependent cellular cytotoxicity. This is mediated by Fcγ receptors on immune cells, particularly FcγRIIIa (CD16) on natural killer … Abstract Background: Rituximab-mediated B-cell depletion in pemphigus patients is majorly caused by antibody-dependent cellular cytotoxicity. This is mediated by Fcγ receptors on immune cells, particularly FcγRIIIa (CD16) on natural killer (NK) cells. This study investigates the impact of FcγRIII expression on response to rituximab in pemphigus patients. Patients and Methods: A cross-sectional study was carried out on 85 pemphigus patients who had been treated with rituximab using a rheumatoid arthritis protocol. Patients who achieved complete remission within 4.5 months were arbitrarily defined as responders. CD16 expression on NK cells, NK-T cells, and monocytes, as well as FcγRIIIa-158V/F polymorphism, were assessed using flow cytometry and genetic assays, respectively. Results: CD16 expression on NK cells and NK-T cells was higher in responders than in resistant patients but did not reach statistical significance. A significant negative correlation was observed between NK-T cell percentage and time to remission ( P < 0.001), suggesting NK-T cell percentage as a possible marker for prediction of time to remission. Distribution of FcγRIIIa genotypes (V/V, V/F, F/F) did not significantly differ between responders and resistant patients ( P = 0.574). A statistically non-significant trend suggested that FF homozygotes may have a slightly worse survival outcome in achieving remission compared to FV/VV genotypes, with a hazard ratio of 1.255 (95% CI: 0.6112–2.577). Limitations: Small, single-center sample, potential selection bias, and lack of long-term follow-up or functional assays. Conclusion: This study underscores the potential influence of CD16 expression and FcγRIIIa polymorphism in response to rituximab in pemphigus patients, emphasizing the need for further research to establish definitive relationships for personalized treatment strategies.

Technique of Conjunctival Biopsy and Direct Immunofluorescence for Diagnosing Mucous Membrane Pemphigoid

2025-06-17

T. Subha Mastan Rao , Sayan Basu , Swati Singh | Journal of Visualized Experiments

Urticarial Plaques With Vesicles in a Young Hispanic Woman

2025-06-16

Valeria Olvera‐Rodríguez , Gerardo González‐Martínez , Sonia Chávez‐Álvarez +4 more | JEADV Clinical Practice

Association Between HLA Class II Gene Polymorphisms and Cytokine Levels in PLWH with HIV-Related Dermatoses in Latvia

2025-06-15

Alena Soha , Inga Ažiņa , Maksims Zolovs +5 more | Medicina

Background and Objectives: This study explores the immunogenetic associations of human leukocyte antigens (HLAs) and cytokine levels in people living with HIV/AIDS (PLWH) who exhibit HIV-related skin disorders. HIV-related skin … Background and Objectives: This study explores the immunogenetic associations of human leukocyte antigens (HLAs) and cytokine levels in people living with HIV/AIDS (PLWH) who exhibit HIV-related skin disorders. HIV-related skin disorders, including inflammatory eruptions, atopic and seborrheic dermatitis, psoriasis, and pruritic papular eruption, are common among PLWH. These conditions may be influenced by genetic and immunological factors. This study aims to investigate the associations between HLA class II alleles, cytokine levels, and the presence of HIV-related dermatoses, providing insights into genetic susceptibility and immune mechanisms. Materials and Methods: This study included 115 PLWH with HIV-related skin disorders and a control group of 80 healthy individuals. HLA allele frequencies were analyzed, and cytokine levels (IL-1β, IL-10, IFN-y) were measured in blood samples. Statistical analyses were performed to identify significant differences in allele frequencies and cytokine responses between the groups. Results: Risk alleles (DQB1*0201:0301, OR = 19.4 and DQA1*0101:0501, OR = 4.2) and protective alleles (DRB1*07:13, OR = 0.19, DRB1*01:13, OR = 0.09, DRB1*04:11, OR = 0.07, and DQA1*0501:0501, OR = 0.24) showed statistically significant differences in frequency (p < 0.05) between PLWH and healthy controls. The protective DQA1*0501:0501 allele was associated with elevated levels of IL-1β (p < 0.001, r = 0.79) and IL-10 (p = 0.010, r = 0.63). Increased IL-1β levels may enhance immune responses, while higher IL-10 levels may exert anti-inflammatory effects, potentially reducing susceptibility to HIV-related dermatoses. Regression analysis revealed that IL-1β (Exp(B) = 0.76, p < 0.001) and IFN-γ (Exp(B) = 1.06, p = 0.043) are significant predictors for the likelihood of developing HIV-related dermatoses. An increase in IL-1β levels reduced this likelihood by 24%, while an increase in IFN-γ levels increased it by 6%. Conclusions: The findings emphasize the interaction between HLA class II alleles and cytokine profiles in determining genetic risk and clinical outcomes in PLWH with HIV-related skin disorders. Protective alleles, such as DQA1*0501:0501, may contribute to immune regulation, offering potential targets for therapeutic interventions in PLWH with dermatoses. Our results highlight the importance of IL-1β and IFN-γ as key modulators in the progression of HIV infection and the development of HIV-related dermatoses. Further research is needed to explore the mechanisms underlying these associations, particularly in the Latvian population, to inform targeted therapeutic strategies.

A 10-Year Observational Study on Treatment Approaches in Pemphigus and Pemphigoid

2025-06-15

Cristiana Palmela Pereira , Rui Santos , Leonor Fernandes Ferreira +4 more | Acta Stomatologica Croatica

The Efficacy of Dupilumab in Pemphigus Vulgaris

2025-06-13

Foday Koroma , S. Abu Amara , Mark Lebwohl | International Journal of Dermatology

A Literature Review About Richter’s Syndrome Presented in the Mouth

2025-06-12

Omid Shahbazi , Fereshteh Goudarzi , Ali Jamali | Indian Journal of Otolaryngology and Head & Neck Surgery

GUIDELINES FOR THE SYSTEMIC TREATMENT OF PATIENTS WITH PEMPHIGUS AT THE UNIVERSITY CLINIC FOR DERMATOLOGY – SKOPJE

2025-06-12

Julija Mitrova Telenta , Viktor Simeonovski , Bojana Batkoska Shekutkoska +5 more | Academic Medical Journal

Pemphigus encompasses a group of life-threatening autoimmune bullous diseases characterized by blisters and erosions on the mucous membranes and skin, requiring a standardized approach to diagnosis and treatment. According to … Pemphigus encompasses a group of life-threatening autoimmune bullous diseases characterized by blisters and erosions on the mucous membranes and skin, requiring a standardized approach to diagnosis and treatment. According to current recommendations, the treatment of diseases in this group involves the use of systemic corticosteroids in combination with immunosuppressants (azathioprine and mycophenolate mofetil), as well as biological therapy with rituximab, which has already been approved as a first-line treatment for moderate to severe pemphigus vulgaris in Europe and the USA. These guidelines were developed for the needs of the University Clinic of Dermatology – Skopje, which serves as a referral center for the diagnosis and treatment of pemphigus in the Republic of North Macedonia. They are based on the Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the European Academy of Dermatology and Venereology (EADV). The guidelines include general information about the disease, a system for grading disease severity, and recommendations for treatment and monitoring.

Epidermolysis Bullosa Acquisita due to Epitope Spreading in a Patient With Recurrent Bullous Pemphigoid

2025-06-12

A. Daponte , Tsitlakidou Anastasia , Tsaousi Athanasia +5 more | International Journal of Dermatology

Successful Treatment of Spesolimab in a Haemodialysis Patient With Acutely Flaring Generalised Pustular Psoriasis

2025-06-12

Yasuyuki Fujita , Sota Itamoto , Akihiro Orita +2 more | JEADV Clinical Practice

ABSTRACT The efficacy and safety of biologics in patients with psoriasis and end‐stage renal disease is rarely reported. Here, we report a case of generalised pustular psoriasis (GPP) in a … ABSTRACT The efficacy and safety of biologics in patients with psoriasis and end‐stage renal disease is rarely reported. Here, we report a case of generalised pustular psoriasis (GPP) in a patient under haemodialysis successfully treated with spesolimab, with a dramatically positive response and excellent tolerance. This case report suggests that spesolimab is a good candidate for treating GPP, even in patients with haemodialysis.

PEMPHIGUS VULGARIS. EXPERIENCE IN DIAGNOSIS, TREATMENT AND PREVENTION

2025-06-10

Н. Н. Лебедев , Steve Marchenko , S.A. Vilezhaninov +1 more | Bulletin of the Medical Institute of Continuing Education

Background. Patient N., 26 years old, complained of painful erosions on the mucous membrane of the mouth, lips, rashes on the scalp and skin of the trunk. On examination: pallor … Background. Patient N., 26 years old, complained of painful erosions on the mucous membrane of the mouth, lips, rashes on the scalp and skin of the trunk. On examination: pallor of the skin of the face, erosions on the mucous membrane of the lips and marginal gingiva, bleeding when touched; marginal gingiva with signs of desquamation; erosion with fragments of epithelium in the soft palate; positive Nikolsky’s sign. The patient was examined, confirming the pemphigus vulgaris (PV) . The treatment led to an improvement in the patient's condition. The patient was discharged under the supervision of a dermatovenerologist at his place of residence with recommendations on reducing the dose of prednisone and dietary intake. The presented clinical case showed difficulties in the diagnosis and treatment of PV and the prevention of exacerbations. The authors of the article studied all the main processes associated with the prerequisites and occurrence of pemphigus vulgaris in men. The methods of diagnosis and differential diagnosis of PV were highlighted. The identified pemphigus vulgaris in the clinical case was examined from the point of view of diagnosis, treatment and prevention of the disease. Purpose. To review a complex clinical case and familiarize medical professionals with such a rare and complex disease as pemphigus vulgaris. Materials and methods. The 26-year-old patient, chosen for the case, was initially misdiagnosed and improperly treated. As a result of the work of highly qualified dentists and dermatovenerologists, the diagnosis was correctly made and adequate treatment was prescribed, leding to improvement in the patient's condition. Results. The patient's condition steadily improved as a result of adequate treatment. Conclusion. A correct and timely diagnosis and conducted treatment, including supportive therapy, are the key to preserving the life and working capacity of patients.