Epidermolysis bullosa acquisita

Abstract Epidermolysis bullosa acquisita is a rare autoimmune disease, characterized by the synthesis of anti-collagen VII autoantibodies, the main component of hemidesmosome anchoring fibrils. The antigen-antibody binding elicits a complex inflammatory response, which culminates in the loss of dermo-epidermal adhesion of the skin and/or mucous membranes. Skin fragility with bullae, erosions, and milia in areas of trauma characterizes the mechanobullous form of the disease. In the inflammatory form of epidermolysis bullosa acquisita, urticarial inflammatory plaques with tense bullae, similar to bullous pemphigoid, or mucosal lesions can determine permanent scars and loss of functionality in the ocular, oral, esophageal, and urogenital regions. Due to the similarity of the clinical findings of epidermolysis bullosa acquisita with other diseases of the pemphigoid group and with porphyria cutanea tarda, the diagnosis is currently confirmed mainly based on the clinical correlation with histopathological findings (pauci-inflammatory subepidermal cleavage or with a neutrophilic infiltrate) and the demonstration of the presence of anti-collagen VII IgG in situ by direct immunofluorescence, or circulating anti-collagen VII IgG through indirect immunofluorescence and/or ELISA. There is no specific therapy for epidermolysis bullosa acquisita and the response to treatment is variable, usually with complete remission in children and a worse prognosis in adults with mucosal involvement. Systemic corticosteroids and immunomodulators (colchicine and dapsone) are alternatives for the treatment of mild forms of the disease, while severe forms require the use of corticosteroid therapy associated with immunosuppressants, intravenous immunoglobulin, and rituximab.

Autoimmune bullous diseases in pregnancy: clinical and epidemiological characteristics and therapeutic approach

Abstract Autoimmune bullous dermatoses are a heterogeneous group of diseases with autoantibodies against structural skin proteins. Although the occurrence of autoimmune bullous dermatoses during pregnancy is low, this topic deserves attention, since the immunological and hormonal alterations that occur during this period can produce alterations during the expected course of these dermatoses. The authors review the several aspects of autoimmune bullous dermatoses that affect pregnant women, including the therapeutic approach during pregnancy and breastfeeding. Gestational pemphigoid, a pregnancy-specific bullous disease, was not studied in this review.

Diagnosis and treatment of skin diseases manifesting as desquamative gingivitis / 中华皮肤科杂志

Skin diseases manifesting as desquamative gingivitis (DG) can be divided into recurrent DG-and chronic DG-related skin diseases,including oral lichen planus,mucosal pemphigoid,pemphigus vulgaris and so on.A thorough medical history,detailed oral and histopathological examinations and serum immunological tests can be helpful for correct diagnosis of DG-related skin diseases.The treatment of DG-related skin diseases includes topical and systemic therapies.It is necessary to individualize treatment protocols due to treatment response.During the treatment of DG,oral hygiene should be strengthened,secondary fungal and bacterial infections should be avoided,and attention should be paid to the protection of oral cavity and periodontal tissues.

Acute renal failure in a patient with epidermolysis bullosa acquisita

Abstract: Epidermolysis bullosa acquisita is a severe autoimmune subepidermal bullous disease. In this report, we described for the first time a patient with epidermolysis bullosa acquisita who developed acute renal failure. There is a possibility that epidermolysis bullosa acquisita and acute renal failure's pathogenesis shared some common autoimmune pathways. Moreover, acute blood volume reduction may be another cause of prerenal kidney failure. Further studies are needed to verify our hypothesis.

Fenômeno de epitope spreading: caracterização clínico imunológica em pacientes portadores de dermatoses bolhosas autoimunes / Epitope spreading" phenomena: clínical and immunopathological characterization in patients with bullous dermatosis

As dermatoses bolhosas autoimunes são um grupo heterogêneo de afecções da pele e/ou mucosas associadas à produção de autoanticorpos dirigidos às moléculas de adesão epitelial. Podem ser classificadas em dermatoses bolhosas intraepidérmicas (pênfigos) ou subepidérmicas (penfigóides, epidermólise bolhosa adquirida). Nos últimos anos, a transição entre dermatoses bolhosas autoimunes ou coexistência de autoanticorpos de diferentes dermatoses têm sido relatadas em alguns pacientes e atribuída ao fenômeno de epitope spreading (ES): a diversificação de epítopos reconhecidos pelo sistema imune evocaria uma reação secundária a antígenos distintos e não relacionados aos da doença primária. Neste trabalho avaliamos a ocorrência de fenômenos de ES em pacientes portadores de pênfigo. CASUÍSTICA E MÉTODOS: Inicialmente, foi realizada análise de dados clínicos e laboratoriais (exame histopatológico, de imunofluorescência direta-IFD, indireta IFI e ELISA) de 351 pacientes portadores de pênfigos acompanhados no Ambulatório de dermatoses bolhosas autoimunes do Departamento de Dermatologia da Faculdade de Medicina da Universidade de São Paulo no período de dezembro de 2002 a dezembro de 2012. Foram selecionados pacientes com quadro sugestivo de conversão à dermatose bolhosa distinta da doença primária. RESULTADOS: Nove pacientes apresentaram sinais sugestivos de fenômeno de ES e foram incluídos no estudo: 8 com a conversão de Pênfigo vulgar (PV) a foliáceo (PF) 2,3% (grupo1) e um de PF a Epidermólise bolhosa adquirida (EBA) 0,3% (grupo 2). No grupo 1 o intervalo mediano para a conversão foi de 3,5 anos. Cinco pacientes apresentaram modificação histopatológica de clivagem intraepidérmica na camada suprabasal para clivagem na camada subcórnea durante a suspeita de ES; 2 apresentaram clivagem na camada epidérmica média durante a transição e um manteve clivagem suprabasal, apesar de quadro clínico sugestivo de PF. Todos os pacientes apresentavam...

Autoimmune bullous skin diseases represent a heterogeneous group of disorders of skin and mucosa associated with autoantibodies against distinct adhesion molecules. They can be classified, based on the level of loss of adhesion in intraepidermal and sub epidermal dermatosis. The shift from an autoimmune blistering disease to another has been recently described and attributed to the "epitope spreading" (ES) phenomena. It occurs when a primary inflammatory/autoimmune process releases "hidden" epitopes which are recognized by the lymphocytes and evoke a secondary reaction to antigens distinct from, and non-cross-reactive, with the disease causing-epitope. This study attempted to characterize the occurrence of ES in pemphigus patients. METHODS: We analyzed data from 351 pemphigus patients treated ambulatorially at the Department of Dermatology, Faculty of Medicine, University of São Paulo, from December 2002 to December 2012. A careful search for clinical and laboratorial (histopathology, direct-DIF and indirect-IIF immunofluorescence, ELISA) changes suggestive of shift to a secondary bullous disease was performed. RESULTS: Nine out of 351 patients presented clínical shift and were included in the study: eight from pemphigus vulgaris (PV) to foliaceus (PF) 2.3% (group 1) and one from PF to epidermolysis bullosa acquisita (EBA) 0.3% (group 2). In group 1, median interval of disease shift was 3.5 years. Of 8 patients with clinical PF, five showed change of histopathology pattern from suprabasilar cleavage to subcorneal acantholysis, two had cleavage within the middle epidermal layer, and one sustained the suprabasilar acantholysis. One shifted back to PV after clinical and histopatological changes of PF. All patients showed intercellular IgG and/or C3 deposits during PV and PF diagnosis by DIF. IIF titers varied from 1:160 to 1:5120. ELISA index for Dsg1 varied from 22 to 319; and for Dsg3 from 0.4 to 224 (positive if > 20)....

A Case of Epidermolysis Bullosa Acquisita Associated with Psoriasis / 대한피부과학회지

Epidermolysis bullosa acquisita (EBA) is an autoimmune subepidermal blistering disease characterized by the presence of circulating IgG autoantibodies to type VII collagen. Various types of autoimmune blistering disease have been reported in association with psoriasis. A 58-year-old man with a 5-year history of psoriasis vulgaris presented with painful and mildly pruritic erythematous multiple bullae and vesicles. Histopathologically, there was a subepidermal bulla with infiltration of inflammatory cells composed of neutrophils and eosinophils. The salt-split skin indirect immunofluorescence test showed IgG binding to the dermal side of the separation, and immunoblotting using normal human dermal extract revealed antibodies directed against a 290-kDa polypeptide. He was diagnosed with EBA and started medication of oral prednisolone and mycophenolate mofetil. Skin lesions were continuously regressed. Of all the autoimmune blistering diseases coexisting with psoriasis, bullous pemphigoid is the most frequent. However, a few cases of EBA associated with psoriasis have been reported in the literature. We report a rare case of EBA coexisting with psoriasis vulgaris.

Immunofluorescence testing in the diagnosis of autoimmune blistering diseases: overview of 10-year experience

BACKGROUND: Immunofluorescence testing is an important tool for diagnosing blistering diseases. OBJECTIVE: To characterize the immunofluorescence findings in patients diagnosed with autoimmune blistering skin diseases. METHODS: We retrospectively analyzed immunofluorescence results encompassing a 10-year period. RESULTS: 421 patients were included and divided into 2 groups: group 1- intraepidermal blistering diseases (n=277) and 2- subepidermal blistering diseases (n=144). For group 1, positive DIF findings demonstrated: predominance of IgG intercellular staining (ICS) and C3 for pemphigus foliaceus-PF (94% and 73% respectively), pemphigus vulgaris-PV (91.5%-79.5%) and paraneoplastic pemphigus-PNP (66%-33%); ICS IgA in 100% of IgA pemphigus cases, and IgG deposits in the basement membrane zone (BMZ) along with ICS in one Hailey-Hailey patient. The IIF findings revealed mean titers of 1:2.560 for PV and 1:1.280 for PF. For paraneoplastic pemphigus, IIF was positive in 2 out of 3 cases with rat bladder substrate. In group 2, positive DIF findings included multiple deposits at basement membrane zone for epidermolysis bullosa acquisita-EBA (C3-89%,IgG-79%,IgA-47%,IgM-21%) mucous membrane pemphigoid-MMP (C3,IgG,IgA,IgM-80%) and bullous pemphigoid-BP (C3-91%,IgG-39%,IgA-11%,IgM-6%), and IgA at basement membrane zone for IgA linear disease (99%) and dermatitis herpetiformis-DH (dermal papillae in 84.6%). For lichen planus pemphigoides, there was C3 (100%) and IgG (50%) deposition at basement membrane zone. indirect immunofluorescence positive findings revealed basement membrane zone IgG deposits in 46% of BP patients, 50% for EBA, 15% for IgA linear dermatosis and 50% for LPP. Indirect immunofluorescence positive results were higher for BP and EBA with Salt-Split skin substrate. CONCLUSION: Our results confirmed the importance of immunofluorescence assays in diagnosing autoimmune blistering diseases, and higher sensitivity for indirect ...

Detection of anti-type Ⅶ collagen antibodies using enzyme-linked immunosorbent assay in the auxiliary diagnosis of epidermolysis bullosa acquisita: a case report and literature review / 中华皮肤科杂志

Objective To evaluate the performance of anti-type Ⅶ collagen antibody detection using enzyme-linked immunosorbent assay (ELISA) in the auxiliary diagnosis of a case of epidermolysis bullosa acquisita (EBA),and to analyze its sensitivity and specificity for the diagnosis of EBA in large-scale studies by review of relevant literature.Methods Serum samples were collected from a patient with typical clinical,histological and immunological manifestations of EBA,4 healthy human controls,5 patients with bullous pemphigoid and 3 patients with pemphigus vulgaris.ELISA was performed to determine the serum levels of anti-type Ⅶ collagen antibodies.Literature regarding the diagnosis of EBA using ELISA was reviewed with a summary of inclusion criteria for patient enrollment,coating antigens,sensitivity and specificity.A comparison was carried out between ELISA and the other serological methods.Results Anti-type Ⅶ collagen antibodies were detected by ELISA in the serum of the patient with EBA (136 U/ml),but not in the other serum samples.The sensitivity and specificity of ELISA in the diagnosis of EBA differed in different studies,but in general,the accuracy of ELISA was higher than that of immunoblotting.Conclusion ELISA is a simple and convenient tool for the diagnosis of EBA with high accuracy.

Localized Epidermolysis Bullosa Acquisita Limited to the Face / 대한피부과학회지

No abstract available.

Accuracy of indirect immunofluorescence on sodium chloride-split skin in the differential diagnosis of bullous pemphigoid and epidermolysis bullosa acquisita.

Background: Previous reports have shown that indirect immunofluorescence (IIF) performed on sodium chloride-split skin (SSS) is helpful to differentiate epidermolysis bullosa acquisita (EBA) from bullous pemphigoid (BP). Antibodies of BP may bind to the epidermal side of SSS, while antibodies of EBA bind to the dermal side. Aims: To determine the accuracy of IIF-SSS in the differential diagnosis of EBA and BP utilizing immunoblotting (IB) analysis. Methods: Sera from 78 patients, diagnosed with BP by clinical features, histopathology, and direct immunofluorescence (DIF), were assayed using IIF-SSS and IB. Results: Of the 43 serum samples with an epidermal reaction to IIF-SSS assay, 42 were recognized with BP antigens (180 kDa or 230 kDa). Of the 11 serum samples with a dermal reaction pattern, 7 were recognized with the 290 kDa antigen of EBA and 3 with sera bound BP antigens. Seven serum samples with epidermal and dermal combined staining, of which 5 of them reacted with BP antigens, 1 reacted with both BP and EBA antigens. One serum sample from each group showed a negative result by IB. Approximately 9.0% (7/78) of patients diagnosed with BP using regular methods were actually EBA. Conclusions: Epidermal reaction using the IIF-SSS assay highly correlated with the diagnosis of BP. However, dermal reactions correlated poorly with EBA, with some serum samples from BP patients binding to dermal-side antigens. In both epidermal and dermal stained sera using IIF-SSS, there was a possibility of BP and EBA. Differential diagnosis should be confirmed using IB, especially in cases of dermal and double staining patterns assayed using IIF-SSS.

Epidermólise bolhosa adquirida inflamatória: relato de caso / Inflammatory epidermolysis bullosa acquisita: case report

Apresenta-se caso de epidermólise bolhosa adquirida inflamatória. Paciente do sexo masculino, 53 anos, há seis meses com erupção vesicobolhosa pruriginosa sobre base eritematosa no tronco, axilas e membros. O exame anatomopatológico mostrou bolha subepidérmica com neutrófilos. A imunofluorescência direta revelou depósitos lineares de IgG, IgA, IgM e C3 na zona da membrana basal, sendo a imunofluorescência indireta e o Salt Split Skin indireto negativos. Anticorpos antinucleares não reagentes. Houve melhora do quadro com prednisona e cicatrização de algumas lesões com formação de milia. Trata-se de apresentação rara de epidermólise bolhosa adquirida, com lesões iniciais predominantemente inflamatórias.

We report a case of an inflammatory variant of epidermolysis bullosa acquisita in a 53-year-old male, with itching blistering eruption on the trunk, armpits and limbs for six months. The skin biopsy specimen showed subepidermal blister with neutrophils. Direct immunofluorescence revealed linear depositions of IgG, IgA, IgM and C3 at the basement membrane; indirect immunofluorescence and salt Split Skin were negative. Antinuclear antibodies were also negative. Improvement of the blisters followed treatment with systemic corticotherapy and some lesions healed with milia. This is a rare presentation of epidermolysis bullosa acquisita, with inflammatory lesions at first.

Successful Treatment of Localized Epidermolysis Bullosa Acquisita with Low-dose Dapsone and Topical Tacrolimus / 대한피부과학회지

A 52-year-old man had a twenty-five year history of recurrent bullous eruption that was localized to both cheeks. The diagnosis of epidermolysis bullosa acquisita was confirmed by means of direct immunofluorescence and salt-split direct immunofluorescence studies that were performed on the perilesional skin. The patient has been in partial remission state with the treatment of low dose dapsone (12.5~25 mg) and topical tacrolimus. Herein, we report on a case of EBA localized to the face, and it showed a favorable response to treatment with low-dose dapsone and topical tacrolimus.

An Ultrastructural Examination of the Epitope Expression at Each Domain of Type VII Collagen in Patients with Epidermolysis Bullosa Acquisita / 대한피부과학회지

BACKGROUND: Epidermolysis bullosa acquisita (EBA) is an autoimmune disease characterized by circulating IgG autoantibodies which bind to the type VII collagen (C-VII). The major antigenic epitopes in C-VII, to which most EBA autoantibodies react, have been considered to be present in the N-terminal noncollagenous (NC1) domain. However, a novel EBA subgroup was recently identified with circulating antibodies, which target domain(s) other than or along with the NC1 domain of C-VII. These data suggest that there might be some heterogeneity in the autoantibody specificity to bind the domain-oriented epitopes in EBA. OBJECTIVE: The purpose of this study was to determine whether additional or independent epitopes exist in the C- terminal noncollagenous (NC2) and/or collagenous triple-helical (CTH) domain among Korean patients with EBA. METHODS: For this investigation, postembedding, indirect, immunogold electron microscopy was performed with the sera from 10 cases of EBA, having circulating autoantibodies against C-VII. The identification of the epitope and the relevant domain in each case were determined by ultrastructural localization of the immunogold particles. RESULTS: From 10 sera examined, all 10 cases showed deposits of gold particles confined to the area along the lamina densa (LD), without any other pattern of deposition. There was no case which revealed any independent/distinct deposits of the gold particles in the dermis below the LD. The ultrastructural locations of each domain (NC1, on the LD; NC2, 300~360 nm below the LD; CTH, between the area of NC1 and NC2) indicated that the epitopes recognized in all 10 Korean cases of EBA were expressed at the NC1 domain of C-VII. We did not find any additional or independent epitope in other domain. CONCLUSION: The results suggest that there may not be a wide heterogeneity in the domain-oriented topographic expression of antigenic epitopes in EBA; it is highly likely that the major epitopes present in Korean EBA cases reside within the NC1 domain of C7, similar to those observed with white population.

Application of Confocal Laser Scanning Microscopy to the Differential Diagnosis of Bullous Pemphigoid and Epidermolysis Bullosa Acquisita / 대한피부과학회지

BACKGROUND: The differential diagnosis of bullous pemphigoid (BP) and epidermolysis bullosa acquisita (EBA) presents some difficulties since both diseases have overlapping clinical and histological features, as well as immunopathological findings. Confocal laser scanning microscopy (CLSM) has been developed and has shown to be a promising tool for dermatological investigations, giving a higher degree of resolution and available co-localization analysis. OBJECTIVE: The purpose of this study was to evaluate whether the new technique of CLSM could reliably identify and differentiate the binding sites of disease specific-autoantibodies (Abs) at the basement membrane zone (BMZ), with the sera from BP and EBA. METHODS: An indirect immunofluorescence (IF) assay was performed to localize the binding sites of circulating Abs from 5 cases of both BP and EBA, as well as the sites of 3 BMZ markers (integrin beta4, laminin-5, and type IV collagen). To facilitate identification and topographic differentiation between the two groups, patients' Abs were labeled with fluorescein isothiocyanate, whereas the BMZ markers were labeled with Texas red. The tissue specimens were observed under both conventional IF microscopy and CLSM. RESULTS: Owing to superposition of antigens and marker labels, double immuno-labeled sections under IF microscopy revealed limitations for the differentiation of patient's sera from BMZ markers even with high magnification (x1,000). However, CLSM was able to eliminate much of the antigen overlap. In BP, the circulating autoantibody' deposits were recognized on the epidermal side of laminin-5 and type IV collagen, and codistributed with integrin beta4. On the other hand, the binding of autoantibodies in EBA was on the dermal side from that of integrin beta4, laminin-5 and type IV collagen. These spatial relationships are compatible with their known microstructural locations. CONCLUSION: Our study indicates that CLSM examination may provide more precise localization of the antigens in BP and EBA than conventional IF microscopy. CLSM would not only be an efficient tool to identify circulating anti-BMZ autoantibodies for diagnosis and differential diagnosis of blistering diseases, but also a great addition to examining tissue specimens in patients who do not have detectable circulating Abs.

Possible Apoptotic Mechanisms in the Vesiculation Process of Pemphigus, Bullous Pemphigoid, and Epidermolysis Bullosa Acquisita / 대한피부과학회지

BACKGROUND: Apoptosis, or programmed cell death, may participate with pathogenesis of intercellular detachment and loss of cell-matrix interaction. Autoimmune bullous dermatoses is an entity charaterized by bullous lesions of the skin and mucosa, and autoantibodies to the specific tissue components. OBJECTIVE: The purpose of this study was to investigate the induction of apoptosis in the lesional skin of pemphigus vulgaris (PV), pemphigus foliaceus (PF), bullous pemphigoid (BP), and epidermolysis bullosa acquisita (EBA). METHODS: Hoechst 33342 (bisbenzimide) staining and terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick end labeling (TUNEL) staining was performed to determine the induction of apoptosis in the lesional skin of each disease. RESULTS: In PV and PF, typical findings of apoptosis were observed in the lesional epidermis showing acantholysis. However, in BP and EBA, no apoptosis of the epidermis was observed. CONCLUSION: These results suggest that apoptosis is only associated with acantholysis of the epidermal keratinocytes, one of many components of pathogenesis in bullous disease, in patients with pemphigus.

A Case of Epidermolysis Bullosa Acquisita with Pseudomembranous Esophageal Involvement / 대한피부과학회지

Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal blistering disorder characterized by autoantibodies to anchoring fibril (type VII) collagen. EBA is known to have a wide clinical spectrum that includes a non-inflammatory mechanobullous presentation, an inflammatory vesiculobullous eruption akin to bullous pemphigoid, and a mucosal centered disease with scarring that is reminiscent of cicatrical pemphigoid. Patients with EBA often have lesions on the oral mucosa, but esophageal involvement has not been well documented. We report a case of EBA in a 63-year-old woman who had a pseudomembranous esophageal lumen. To our knowledge, this is the first report of EBA with pseudomembranous esophageal involvement.

A Case of Epidermolysis Bullosa Acquisita (Cicatricial pemphigoid-like type) / 대한피부과학회지

Epidermolysis bullosa acquisita (EBA) is an uncommon autoimmune subepidermal blistering disorder and has four clinical subtypes. Among the four types of EBA, the cicatricial pemphigoid-like type is rarer than the other types and clinically the worst one. We experienced a case of cicatricial pemphigoid-like type of EBA in a 69-year-old woman, whose initial symptom was painful erosive lesions of oral mucous membrane before development of ocular and bullous cutaneous lesions. The clinical, histopathological findings and immunoblot assay were all typical of the disease. The course of her disease showed remissions by treatments including corticosteroid and intravenous immunoglobulin, but each time with exacerbations.

Two Cases of Epidermolysis Bullosa Acquisita with Atypical Distribution of Eruptions / 대한피부과학회지

We herein report two patients with epidermolysis bullosa acquisita(EBA), who had showed the atypical clinical features. A 25-year-old male presented with multiple pruritic vesicles, erosion and crusts which occurred more severely on the face than any other sites of the skin and healed with atrophic scar formation. Histopathologically, there was a subepidermal neutrophilic blister with moderate perivascular lymphohistiocytic infiltrates in the dermis, and the diagnosis of EBA was confirmed by means of direct immunofluorescence and salt-split direct immunofluorescence test performed on the perilesional skin. The other patient was a 24-year-old female who had had multiple painful ulcerative lesions on the oral mucosa for 4 months. After then, vesicles and bullae developed to progressively generalize to the anterior neck, chest and lower leg. Histolopathologic examination of peribullous skin showed a subepidermal bulla with neutrophils and eosinophils. The salt-split skin direct immunofluence test showed IgG and IgM binding to the dermal side only. We diagnosed this patient as nonscarring inflammatory EBA. Both patients were treated with prednisolone, colchicine, and dapsone resulting in clinical improvement, but their skin lesions recurred several months after discontinuing medication.

Epidermolysis Bullosa Acquisita: A Complete Remissions versus Patients with Long-term Persistent Activities / 대한피부과학회지

BACKGROUND: In epidermolysis bullosa acquisita, it has been recognized that there exists heterogeneity in the clinical and serologic/immunopathologic features. OBJECTIVE: We examined patients with epidermolysis bullosa acquisita to see if there were any associated clinical and serological features which may predict disease activity or prognosis in the disease. METHODS: Clinical and some serologic features were compared. between 2 groups of patients with epidermolysis bullosa acquisita; one with complete remission of the symptoms and signs of the disease for more than 2 years and the other group with persistent disease activities of longer than 5 years.

Subepidermal Blistering Diseases with Coexistent IgG and IgA Anti-BMZ Antibodies: Epidermolysis and Cicatricial Pemphigoid / 대한피부과학회지

A 21 year-old lady had erythemas and bullae on the trunk for 20 days. Biopsy specimens showed subepidermal blister and deposits of IgG and IgA along the basement membrane zone (BMZ). Her serum antibodies of IgG and IgA were observed to recognize 290/145 kD antigens derived from A431-cell line. The other patient of 62 year-old woman had recurrent oral erosions and erosio-ulcerative plaques on her anterior chest for the past 2 years. Tissue specimens showed subepidermal blister and deposits of IgG and IgA at the BMZ. The patients serum had IgG and IgA antibodies which had binding specificities to the 230 kD pemphigoid antigen. The above two cases of epidermolysis billosa acquisita and cicatricial pemphigoid with IgG and IgA antibodies of comparable titers in each may be rarely encountered ones.