Comparative study of pemphigus vulgaris and pemphigus foliaceus in Singapore

This is a retrospective study of all patients diagnosed to have pemphigus in our centre over a 3 year period. The case records of all patients with pemphigus from January 1995 to December 1997 were analysed. Fifty patients were diagnosed to have pemphigus during the study period. The diagnoses were pemphigus vulgaris in 31 patients, pemphigus foliaceus in 16, paraneoplastic pemphigus in two and IgA pemphigus in one. The average titre of anti-intercellular antibodies in patients with pemphigus vulgaris (1:96) was higher than the titre in patients with pemphigus foliaceus (1:69). The average initial dose of prednisolone required for disease control in patients with pemphigus vulgaris (62 mg/day) was significantly higher than that required for patients with pemphigus foliaceus (44 mg/day). In our study population, pemphigus vulgaris is a more severe and chronic disease than pemphigus foliaceus, as reflected in the higher titre of anti-intercellular antibodies, higher dose of systemic corticosteroids required for control of the disease, the longer duration to achieve complete remission and longer follow-up period.     

Comorbid mental health issues in patients with pemphigus vulgaris and pemphigus foliaceus

The term ‘pemphigus’ refers to chronic autoimmune skin disorders that cause blistering erosions on the skin and oral mucosa. The two major clinical forms are pemphigus vulgaris and pemphigus foliaceus. Although rare, they confer a stark symptomatic burden upon patients that significantly impacts daily life. Comorbid mental health issues are not routinely screened for in patients with pemphigus, and current UK guidance provides no formal provision for the identification and treatment of psychological issues. This review is the first of its kind, to our knowledge, to systematically examine the available evidence on mental health issues in pemphigus. Published work suggests that the incidence of anxiety and depression is much higher in patients with pemphigus compared with both the general population and with patients having other chronic skin disorders. Disease severity appears to be closely linked to mental health, with worsening of pemphigus associated with deteriorations in psychological wellbeing. Corticosteroids, which are associated with depression in chronic use, are the current first-line therapy for pemphigus and have been identified as a potential confounder and independent risk factor for mental health comorbidity in pemphigus. Current evidence is unclear whether a bidirectional relationship exists between mental health and pemphigus severity, and more thorough research is required to develop understanding of this issue. In conclusion, we have identified a high incidence of mental health comorbidity in pemphigus, and recommend routine screening of patients with pemphigus for mental health issues and signposting toward mental health services as an initial measure to address this.     

Validity of the EQ-5D in patients with pemphigus vulgaris and pemphigus foliaceus

Pemphigus is a group of autoimmune skin diseases that cause blisters and wounds on the skin and/or mucous membranes such as inside the mouth. It is an uncommon disease with around 15 new cases yearly per million people. The two most common clinical forms are pemphigus vulgaris and foliaceus. Pemphigus can still be life threatening, and the symptoms may cause significant difficulties for patients in their everyday life. This study, from Hungary, aimed to assess the health status and quality of life in patients with pemphigus vulgaris and foliaceus. The authors surveyed 109 pemphigus patients, and collected data on demographics and clinical characteristics. Quality of life was assessed by the EQ-5D general health status questionnaire. Overall, 50%, 43%, 43%, 42% and 19% of pemphigus patients reported problems regarding pain/discomfort, mobility, anxiety/depression, usual activities and self-care, respectively. Patients having skin and mucosal symptoms, a larger number of comorbidities (other diseases occurring alongside pemphigus) and more severe disease experienced greater impairment of quality of life. No difference was found in quality of life scores between pemphigus vulgaris and foliaceus patients or between females and males. This study demonstrates that the EQ-5D questionnaire is an accurate measure of quality of life in pemphigus patients. The EQ-5D questionnaire allows comparisons between patients across a broad range of disease areas and people without those diseases. The EQ-5D scores reported in this study can be useful for cost-effectiveness analyses of new pemphigus treatments such as rituximab, and might help to improve patients’ access to more effective medicines, in a condition for which only limited treatment options have been available so far.     

Drug-induced pemphigus: autoantibodies directed against the pemphigus antigen complexes are present in penicillamine and captopril-induced pemphigus

Pemphigus is an autoimmune blistering disease characterized by circulating autoantibodies directed against the keratinocyte cell surface. The two variants, pemphigus foliaceus and pemphigus vulgaris, can be distinguished at the molecular level by immunochemical studies. The large majority of patients with pemphigus develop the disease spontaneously; however, there is a small group of patients who develop pemphigus after treatment with certain medications, of which penicillamine and captopril are the best documented. Most patients with drug-induced pemphigus have circulating and/or tissue bound epidermal cell surface autoantibodies; however, the molecular specificity of these autoantibodies has not been studied. We performed immunoprecipitation studies utilizing extracts of 125I-labeled suction blister epidermis and the sera of three patients with drug-induced pemphigus foliaceus (two due to penicillamine and one due to captopril) and one patient with captopril-induced pemphigus vulgaris. We found that the three patients with drug-induced pemphigus foliaceus had circulating autoantibodies that are directed against the pemphigus foliaceus antigen complex and that the one patient with drug-induced pemphigus vulgaris had circulating autoantibodies that are directed against the pemphigus vulgaris antigen complex. This study demonstrates that autoantibodies from drug-induced pemphigus patients have the same antigenic specificity, on a molecular level, as do autoantibodies from other pemphigus patients.     

Childhood pemphigus

BACKGROUND: Five children with pemphigus are reported: three with pemphigus vulgaris, one with pemphigus vegetans, and one with pemphigus foliaceus. Only one case of juvenile pemphigus vegetans has been published in the literature. MATERIALS AND METHODS: All three patients with pemphigus vulgaris were treated with oral corticosteroid; in two cases, azathioprine was added for steroid-sparing effect. The patient with pemphigus vegetans had a clinical presentation resembling pemphigus vulgaris, but the lesions in the perianal area healed as hypertrophic granulation tissue. He was treated with oral corticosteroid, azathioprine, and intralesional corticosteroid. The patient with pemphigus foliaceus presented with exfoliative dermatitis, and was treated with oral corticosteroid; methotrexate was added later for steroid-sparing purposes RESULTS: The patients were followed up for 1-4 years; the prognosis of childhood pemphigus is good. CONCLUSIONS: Long-term follow-up is needed to detect flaring of the disease and the side-effects of immunosuppressive drugs.     

Treatment of pemphigus vulgaris and pemphigus foliaceus with mycophenolate mofetil

BACKGROUND: Mycophenolate mofetil is increasingly being used as a corticosteroid-sparing agent in immunosuppressive regimens. OBJECTIVE: To elucidate the effectiveness of mycophenolate as adjuvant therapy in the treatment of both pemphigus vulgaris and pemphigus foliaceus. DESIGN: Historical prospective study. SETTING: University hospital. PATIENTS: The study included 42 consecutive patients with pemphigus (31 with pemphigus vulgaris and 11 with pemphigus foliaceus) who had relapses during prednisone taper or had clinically significant adverse effects from previous drug therapy. RESULTS: Remission was achieved in 22 (71%) and 5 (45%) of patients with pemphigus vulgaris and pemphigus foliaceus, respectively. Partial remission was achieved in 1 (3%) and 4 (36%), respectively. The median time to achieve complete remission was 9 months (range, 1-13 months). The treatment was administered for a median of 22 months, and the median follow-up period was 22 months. Seventy-seven percent of patients had no adverse effect. Two patients had side effects severe enough to necessitate discontinuation of treatment, one because of symptomatic but reversible neutropenia and the other because of nausea. CONCLUSION: Mycophenolate is an effective and safe adjuvant in the treatment of both pemphigus vulgaris and pemphigus foliaceus.     

Common human leukocyte antigen alleles in pemphigus vulgaris and pemphigus foliaceus Italian patients.

Pemphigus refers to a group of autoimmune blistering skin diseases, mainly identified as pemphigus vulgaris and pemphigus foliaceus, both characterized by the presence of autoantibodies against keratinocyte adhesion molecules, leading to loss of cell-cell adhesion with consequent blister formation. Pemphigus vulgaris is reported to be associated with human leukocyte antigen DR4 and/or DR6 whereas no data are available on pemphigus foliaceus, except for the endemic Brazilian form (fogo selvagem), which is reported to be associated with DR1 and DR4. We here report human leukocyte antigen molecular typing on a total of 87 patients, 61 with pemphigus vulgaris and 26 with pemphigus foliaceus, versus 128 healthy matched controls. Generic typing showed an increase of DRB1*04 and DRB1*14 and a decrease of DRB1*07 in both pemphigus vulgaris and pemphigus foliaceus patients. Molecular subtyping of DR4+ and DR14+ subjects showed a highly significant association between the DRB1*1401 and both pemphigus vulgaris (p < 0.0001) and pemphigus foliaceus patients (p < 0.0001) together with a significant increase of the linked DQB1*0503 (pemphigus vulgaris p < 0.0001; pemphigus foliaceus p < 0.0001). Moreover, whereas the association between DRB1*0402 and pemphigus vulgaris (p < 0.0001) has been confirmed, no significant association between a specific allele of the DR4 group and pemphigus foliaceus, has been found. Therefore, at least in Italian patients, pemphigus vulgaris and pemphigus foliaceus share DRB1*1401 and DQB1*0503, as susceptible human leukocyte antigen alleles, whereas DRB1*0402 is only found associated with pemphigus vulgaris. The observation that both diseases, pemphigus vulgaris and pemphigus foliaceus, carry the same susceptible human leukocyte antigen alleles has been interpreted as a common genetic background predisposing to pemphigus as, like in other autoimmune disorders, it is not sufficient to explain the onset of the disease on the basis of the sole aforementioned alleles. Other linked genes and/or environmental factors should play a facilitating role in the outbreak of pemphigus, either pemphigus vulgaris or pemphigus foliaceus.     

Quality of life and pemphigus

INTRODUCTION: Pemphigus is a frequent chronic bullous dermatosis in Morocco that predominantly affects young women. It requires serious therapeutic management and often repeated hospitalizations. The purpose of this study was to assess the particular impact of pemphigus on quality of life by comparing the quality of life in patients suffering from pemphigus with that of the general population using a generic instrument of measure of health related quality of life: the SF-36, in association with a questionnaire exploring the impact of the disease on self perception, social relationship and behaviour. PARTICIPANTS AND METHODS: A French version of the SF-36 underwent 2 translations from French to Moroccan dialect, then two retrograde translations. After a pre-test, the questionnaire survey was administered by the same investigator to 30 patients who suffered from pemphigus, followed-up in the Ibn Rochd UHC in Casablanca and to 60 healthy adults. The two samples were paired according to age and sex. RESULTS: In the pemphigus group, there was a significant decrease of mean scores of all the SF-36 dimensions, except for physical pain and alteration in general status of health. The greatest alteration concerned the impact on physical and emotional status (p<0.00001) and the smallest alteration concerned the perception of general health (p=0.02). The factors influencing this impact were: profession, face involvement and extent of lesions. Other results concerning the psychosocial impact of pemphigus are presented. CONCLUSION: Our study confirmed that pemphigus is responsible for great alterations in health related quality of life. Therefore, the management of this disease must take into account its impact on various fields of life of the patients.     

The value of trichoscopy in the differential diagnosis of scalp lesions in pemphigus vulgaris and pemphigus foliaceus

BACKGROUND

Trichoscopy is becoming increasingly popular in diagnosing hair and scalp diseases. Scalp involvement in pemphigus is common. The scalp may be the first or only site of clinical manifestation of the disease.

OBJECTIVE

The aim of this study was to analyze whether trichoscopy may be useful in aiding differential diagnosis of scalp lesions in patients with pemphigus vulgaris and pemphigus foliaceus.

METHODS

Trichoscopy was performed in 19 patients with scalp lesions in the course of pemphigus (9 patients with pemphigus vulgaris and 10 with pemphigus foliaceus). In all patients, the diagnosis of scalp pemphigus was confirmed by histopathology. The working magnification was 20-fold and 70-fold.

RESULTS

The most frequently observed trichoscopy features of pemphigus lesions were: extravasations (18/19; 94.7%) and yellow hemorrhagic crusts (11/19; 57.9%). Yellow dots with whitish halo were observed in 6/19 (31.6%) patients with pemphigus. White polygonal structures were observed in pemphigus foliaceus (6/10; 60%), but not in pemphigus vulgaris. Vascular abnormalities were more frequent in pemphigus vulgaris, when compared to pemphigus foliaceus, and were associated with a severe course of disease. Linear serpentine vessels were the most frequent vascular abnormality in patients with pemphigus vulgaris and pemphigus foliaceus (77.8% and 30%, respectively).

CONCLUSION

Trichoscopy may serve as a useful supplementary method in the differential diagnosis of pemphigus, especially in cases of desquamative or exudative lesions limited to the scalp. Extravasations, yellow hemorrhagic crusts, yellow dots with whitish halo, white polygonal structures and linear serpentine vessels are trichoscopy features which may suggest the diagnosis of pemphigus.     

Oxidative stress in patients with endemic pemphigus foliaceus and healthy subjects with anti-desmoglein 1 antibodies

Background:Previous studies have shown oxidative stress in pemphigus vulgaris and pemphigus foliaceus, nevertheless, it remains unknown whether a similar response is characteristic of endemic pemphigus foliaceus in Peru.Objectives:To determine the oxidative stress response in endemic pemphigus foliaceus patients and subjects with positive for anti-desmoglein1 antibodies (anti-dsg1) from endemic areas of Peru.Subjects and methods:This is a cross-sectional study. The study population included 21 patients with Endemic Pemphigus foliaceus and 12 healthy subjects with anti-dsg1 antibodies from the Peruvian Amazon (Ucayali), as well as 30 healthy control subjects. Malondialdehyde, an indicator of lipid peroxidation by free radicals, was measured in serum.Results:We collected 21 cases of endemic pemphigus foliaceus, 15 of them with active chronic disease and 6 in clinical remission. Serum malondialdehyde values in patients with chronic active evolution and healthy subjects with anti-dsg1 antibodies were statistically higher than those of healthy controls (p<0.001). There was no significant difference between serum values of localized and generalized clinical forms.Study limitations:The main limitation of this present study is the small number of patients with endemic pemphigus and healthy subjects positive for desmoglein 1 antibodies.Conclusions:The increased serum levels of malondialdehyde in patients with chronic active endemic pemphigus foliaceus and healthy subjects from endemic areas with anti-dsg1 antibodies may suggest a contribution of systemic lipid peroxidation in the pathogenesis of endemic pemphigus foliaceus.     

Therapeutic effect of mizoribine on pemphigus vulgaris and pemphigus foliaceus

We evaluated the effectiveness of mizoribine, a newly developed immunosuppressive agent, as an adjuvant therapy in the treatment of both pemphigus vulgaris and pemphigus foliaceus. Eleven pemphigus patients (eight pemphigus vulgaris and three pemphigus foliaceus) received the combination therapy of prednisolone and mizoribine. Complete remission was observed in three of the eight patients with pemphigus vulgaris and in one of the three patients with pemphigus foliaceus. The four patients with complete remission had a rapid clinical response and achieved remission at a median of 11.8 months. Partial remission was achieved in two of the three patients with pemphigus foliaceus. The median time to achieve partial remission was 16.0 months. Six (55.6%) of the 11 patients with pemphigus had complete or partial remission and were able to taper their prednisolone. The cumulative probability of having a complete remission was 64.3% at 19 months of follow-up using Kaplan-Meier analysis. The effectiveness of the additional mizoribine therapy could be attributed to its corticosteroid-sparing properties as well as its immunosuppressive effects. The serum concentration titer of mizoribine was around 1.0?μg/mL 2 hours after administration. Patients who were not improved by the additional mizoribine might require a continuously higher dose of mizoribine to achieve effective therapy.     

History of pemphigus

The modern concept of pemphigus divides it into variants: pemphigus vulgaris, pemphigus vegetans, pemphigus foliaceus, pemphigus erythematosus (Senear-Usher syndrome), and pemphigus herpetiformis. These conditions are all related by the fact that they are all bullous diseases at some time during their clinical course and are histologically characterized by acantholysis. In pemphigus vulgaris and pemphigus vegetans, acantholysis is generally above the basilar cell layer (suprabasilar) or in the lower half of stratum spinosum. In pemphigus foliaceus, pemphigus herpetiformis and pemphigus erythematosus, acantholysis occurs in the granular cell layer or upper stratum spinosum. Autoantibodies to the intercellular cement substance of the epidermis are present in all the variants.

The changes in the concept of pemphigus over time has been reviewed.^1,2 The term “pemphigus,” itself, was first proposed by Boissier de Sauvages in his classification of skin diseases.³ He described pemphigus maior as an acute, febrile, blistering disease lasting only two weeks. Today, the diagnosis would probably be erythema multiforme.

Wichmann^4,5 was the first to describe pemphigus as a chronic bullous disease; he used the term, febris bullosa, to describe bullous eruptions of short duration. Unfortunately, few authors agreed with Wichmann's more restricted concept, and continental dermatologists continued to use the term pemphigus for a wide range of vesicular or bullous diseases. For example, Gilibert,⁶ in his monograph on pemphigus, included almost every disease with bullae in his classification. His “chronic pemphigus” most closely corresponds to the modern concept of pemphigus. Von Martius⁷ had an equally liberal definition of pemphigus, dividing it into 97 types.

Willan had a much more restricted view of the disease. In his classification of skin diseases, pemphigus vulgaris was a febrile, bullous eruption of short duration.⁸ He applied the term “pompholyx duitinus” to a chronic, bullous eruption, without inflammation and fever, similar to the modern concept of pemphigus vulgaris.     

Treatment of severe refractory pemphigus vulgaris with rituximab

Pemphigus vulgaris is a potentially fatal autoimmune bullous disease. High doses of immunosuppressive drugs are used in managing severe cases of pemphigus. Rituximab, an anti-CD20 monoclonal antibody, has proven to be effective in patients with refractory pemphigus vulgaris and pemphigus foliaceus. We review cases of pemphigus vulgaris and pemphigus foliaceus not associated with lymphoma that were treated with rituximab, and we report a new case of severe refractory pemphigus vulgaris successfully treated with rituximab.     

Nonendemic pemphigus foliaceus presenting as fatal bullous exfoliative erythroderma

Pemphigus foliaceus is a cutaneous autoimmune blistering disease that is characterized by lower morbidity and mortality than those observed in pemphigus vulgaris or paraneoplastic pemphigus. However, erythrodermic forms of the endemic variant of pemphigus foliaceus have been associated with a higher mortality. We report a case of nonendemic pemphigus foliaceus that presented as fatal bullous exfoliative erythroderma, and thus, we will emphasize the inclusion of this entity in the differential diagnosis and the use of skin direct immunofluorescence in the evaluation of patients with erythroderma.     

Autoantibodies against desmoglein 2 are not pathogenic in pemphigus

BackgroundAnti-desmoglein 1 and 3 autoantibodies justify acantholysis in pemphigus; however, the pathogenesis of anti-desmoglein 2 is hypothetical.ObjectiveTo compare the participation of desmogleins 1, 2 and 3 through the production of serum autoantibodies, and protein and gene expression in the skin/mucosa of patients with pemphigus foliaceus and pemphigus vulgaris.MethodsThe autoantibodies were titrated by ELISA in 202 samples of pemphigus foliaceus, 131 pemphigus vulgaris, 50 and 57 relatives of patients with pemphigus foliaceus and pemphigus vulgaris, respectively, and 114 controls. Protein and gene expressions were determined by immunohistochemistry and qPCR in the skin/mucosa of 3 patients with pemphigus foliaceus and 3 patients with pemphigus vulgaris.ResultsHigher titers of anti-desmoglein 2 (optical density) resulted in pemphigus foliaceus and pemphigus vulgaris, when compared to controls (0.166; 0.180; 0.102; respectively; p < 0.0001). There was a correlation between anti-desmoglein 2 and anti-desmoglein 1 titers in pemphigus foliaceus (r = 0.1680; p = 0.0206). There was no cross-reaction of anti-desmoglein 2 with desmoglein 1 and 3. Protein overexpression of desmoglein 2 was observed in intact and lesional skin of patients with pemphigus compared to the skin of controls. Internalization granules of desmoglein 1 and 3, but not of desmoglein 2, were observed in lesions of pemphigus foliaceus and pemphigus vulgaris, respectively. Gene overexpression of desmoglein 2 was observed in the mucosa.Study limitationsSmall sample size for the statistical analysis of protein and gene expression.ConclusionAutoantibodies against desmoglein 2 are not pathogenic in pemphigus; protein and gene overexpression of desmoglein 2 in the skin and mucosa may be involved in acantholysis repair.     

Curvicircular intracytoplasmic membranous structures in keratinocytes of pemphigus foliaceus

We noticed intracytoplasmic membranous, annular, or circular structures in the lesion of pemphigus foliaceus and studied these by regular transmission electron microscopy and immuno-electron microscopy. These curvicircular bodies were observed in the preacantholytic keratinocytes of the blister wall as well as in acantholytic cells in 6 out of 6 patients with pemphigus foliaceus. They were absent in samples from 3 patients with pemphigus vulgaris. These structures were about 60–70 nm wide and consisted of 4 electron-dense layers. They were continuous with intact desmosomal structures and gap junctions in the periphery of the keratinocytes. These curvicircular membranous bodies were well labeled with immunogold particles for desmoglein, plakoglobin, connexin 43, and IgG. In contrast to pemphigus vulgaris, splitting of desmosomes through dissolution of intercellular desmoglea was seldom observed in all 6 specimens of pemphigus foliaceus. These findings suggest that in pemphigus foliaceus 1) curvicircular bodies are derived from internalized desmosomes and gap junctions, and 2) cell-to-cell adhesions are weakened by this internalization and acantholysis is initiated, while in pemphigus vulgaris the dissolution of clesmoglea is the initial event. It is suggested that in pemphigus foliaceus the binding of autoantibody induces internalization of many intact desmosomes and gap junctions rather than splitting them.     

Juvenile pemphigus

Seven cases of juvenile pemphigus vulgaris and one case of juvenile pemphigus foliaceus are presented. A detailed review of the literature is also presented. The majority of patients with juvenile pemphigus develop pemphigus vulgaris. The patients present with a wide clinical spectrum. The oral cavity is frequently involved. The mean duration is 4 years. Clinical follow-up is similar to the adult variety. Several patients warrant high doses of systemic corticosteroids and develop serious side effects, most notably growth retardation and opportunistic infections. Short-term judicious use of immunosuppressive agents is advocated. Dapsone is a helpful adjuvant to therapy. It is proposed that relevant immunofluorescent studies be done very early. Early diagnosis and therapy are associated with a better prognosis.     

天疱疮发病机制的研究进展

天疱疮是自身免疫引起表皮棘层细胞松解导致的慢性复发性表皮内大疱性皮肤病,典型表现为红斑基础上的疱壁松弛性水疱、糜烂、尼氏征阳性.依据天疱疮的临床表现,分为寻常型、增殖型、落叶型和红斑型天疱疮.天疱疮主要的发病机制是患者存在针对角质形成细胞桥粒芯(糖)蛋白的自身抗体,但棘层松解的详细机制尚不清.近年来,随着对蛋白组学、免疫学和分子生物学技术的发展以及天疱疮发病机制研究的不断深入,发现天疱疮的发病机制中除传统的桥粒芯(糖)蛋白自身抗体外,非桥粒芯(糖)蛋白抗体因素也参与了棘层松解的形成,为天疱疮提供了新的潜在治疗靶位.     

人皮肤和猴食道上皮用于间接免疫荧光检测天疱疮抗体的比较

目的 :　比较人皮肤 (HS)和猴食道上皮 (MO)用于检测天疱疮抗体的差异及不同底物检测的抗体滴度与疾病严重度的相关性。方法 :　采用HS和MO为底物分别检测天疱疮患者血清中抗体。结果 :以HS和MO为底物检测天疱疮患者血清抗体的阳性率分别为 73.5 %和 87.8% ,而联合两种底物检测的阳性率为 93.9%。两种底物检测PV抗体滴度与疾病严重度均有相关性 (P <0 .0 5 ) ,而PF仅以HS为底物时的抗体滴度与疾病严重度密切相关 (P <0 .0 0 1)。结论 :　MO适于检测PV ,HS适于检测PF ,两种底物联合可提高间接免疫荧光 (IIF)的敏感性。底物的选择对天疱疮病情严重度与抗体滴度的相关性有影响     

Dual diagnosis of Pemphigus and pemphigoid. Retrospective review of thirty cases in the literature.

BACKGROUND: Pemphigus and pemphigoid are two distinct groups of autoimmune blistering diseases. There are many reports of the simultaneous presence of clinical and serological features of both diseases in the same patient. OBJECTIVE: This study is a retrospective review of the present literature on reports of patients with features of both pemphigus and pemphigoid. We recommend that these patients be considered as having a dual diagnosis. METHODS: A review of the English language, peer-reviewed literature was conducted on patients described with features of pemphigus and pemphigoid. Available data on clinical profile, histology, immunopathology, treatment, follow-up and outcome were studied in 30 patients. They were divided into three groups: (1) bullous pemphigoid and pemphigus vulgaris, (2) mucous membrane or cicatricial pemphigoid and pemphigus vulgaris and (3) bullous pemphigoid and pemphigus foliaceus. RESULTS: In all three groups, most patients had a clinical phenotype resembling both diseases. In 17 patients with bullous pemphigoid and pemphigus vulgaris, 83% had a skin biopsy consistent with bullous pemphigoid, 70% had direct immunofluorescence studies typical of bullous pemphigoid and sera of 83% had antibodies typical of pemphigus vulgaris on indirect immunofluorescence. In 10 patients with mucous membrane or cicatricial pemphigoid and pemphigus vulgaris, a histology of mucous membrane pemphigoid was reported in 60% of the patients, direct immunofluorescence studies typical of mucous membrane pemphigoid were reported in 70% of the patients and in 80%, autoantibodies characteristic of pemphigus vulgaris were observed. In 3 patients with bullous pemphigoid and pemphigus foliaceus, the histologies were consistent with bullous pemphigoid, direct immunofluorescence was typical of pemphigus foliaceus and their sera had both autoantibodies. The majority of the 30 patients required long-term high-dose corticosteroids and immunosuppressive agents to control their disease. Three patients with bullous pemphigoid and pemphigus vulgaris (18%) died due to effects of prolonged immunosuppression. CONCLUSION: We characterize a group of patients who have clinical, histological and immunopathological features of bullous or mucous membrane or cicatricial pemphigoid with serological features of pemphigus. These patients did not achieve a prolonged clinical remission by conventional therapy. It is possible that early identification of these patients may improve their prognosis.