Unique Autoimmune Bullous Dermatosis Presenting with <i>in vivo</i> IgG Deposition Both in Intercellular Spaces and along Dermoepidermal Junctions : Report of Five Cases and Analyzes of IgG Subclasses

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  • 表皮細胞間と基底膜部の両部位にIgGの沈着を認めた自己免疫性水疱症の5例―症例報告とIgG subclassesの検討
  • ヒョウヒ サイボウ カン ト キテイ マクブ ノ リョウ ブイ ニ IgG ノ チンチャク オ ミトメタ ジコ メンエキセイ スイホウショウ ノ 5レイ ショウレイ ホウコク ト IgG subclasses ノ ケントウ

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Abstract

We present five cases of unique bullous dermatosis with in vivo IgG deposition both in intercellular spaces and along dermoepidermal junctions in immunofluorecence studies. Cases 1 and 5 had mucosal involvement. Cases 2 and 3 had annular erythema. Case 4 had butterfly rash-like erythema on the cheeks. Histology showed both intraepidermal and subepidermal bullae in Case 1, subepidermal bulla in Cases 2 and 3, and intraepidermal bullae in Cases 4 and 5. In ELISA, anti-Dsg3 antibody and anti-BP180 antibody was positive in Case 1, anti-Dsg1 antibody, anti-Dsg3 antibody, and anti-BP180 antibody, were positive in Case 2, anti-Dsg1 antibody was positive in Case 4, and anti-Dsg3 antibody was positive in Case 5. We diagnosed Case 1 as a combination of pemphigus vulgaris and bullous pemphigoid, Cases 2 and 3 as bullous pemphigoid, Case 4 as pemphigus folliaceus, and Case 5 as pemphigus vulgaris. In conclusion, this unique autoimmune bullous dermatosis is a heterogenous entity, because the clinical features and immunological analyses showed various results. The main IgG subclass in these bullous dermatoses was IgG1.

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