悪性リンパ腫に合併した水疱症

・悪性リンパ腫患者で水疱を伴う多形紅斑，眼・口腔粘膜疹を主訴に当科紹介．Stevens-Johnson（SJS）症候群と自己免疫性水疱症との鑑別を要した．・粘膜型類天疱瘡（mucous membrane pemphigoid：MMP）と診断したが，血液検査で抗デスモグレイン3抗体と抗BP180抗体陽性が判明．interface dermatitis所見に気づき，診断を腫瘍随伴性天疱瘡（paraneoplastic pemphigus：PNP）と変更した．・自験例のように緊満性水疱を呈しNikolsky徴候（N徴候）陰性で直接蛍光抗体法（direct immunofluorescence：DIF）にて基底膜部に強い蛍光がみられ，かつ抗BP180抗体陽性というPNP症例は初報告であった．（「症例のポイント」より）

Bullous disease in a patient with malignant lymphoma Sonoda, Sanae1）Yamanaka, Miki2）Kamae, Tsuyoshi2） 1）Department of Dermatology, Federation of Nationnal Public Service and Affiliated Personal Mutual Aid Association Otemae Hospital 2）Department of Hematology, Federation of Nationnal Public Service and Affiliated Personal Mutual Aid Association Otemae Hospital Abstract We described a patient with paraneoplastic pemphigus (PNP)and B-cell follicular lymphoma. He presented with edematous erythema, vesicles and oral mucosal ulcer, later developing erythema multiforme, skin ulcer, pharyngoesophageal ulcer, pseudomembranous conjunctivitis, and corneal epithelial erosion. Nikolsky sign was negative. Skin biopsy revealed suprabasal acantholytic blister. Direct immunofluorescence showed C3 deposition on the BMZ. In enzyme-linked immunosorbent assays (ELISA), the patient’s serum reacted with Dsg3 and BP180. PNP that has a feature of pemphigoid, is rare.