Guggisberg D, Hohl D
Service de Dermatologie, CHUV, Lausanne, Suisse.
Dermatology. 1995;191(4):352-4. doi: 10.1159/000246600.
We report the case of a 62-year-old patient with an extracutaneous CD30+ anaplastic large T-cell lymphoma which was preceded by intraepidermal IgA pustulosis (IEAP). The clinical picture was identical to Sneddon-Wilkinson disease and histologic examination showed subcorneal pustules with slight acantholysis. Direct immunofluorescence exclusively revealed IgAK deposits on the plasma membrane of suprabasal keratinocytes. Indirect immunofluorescence and immunoelectrophoresis showed no evidence of monoclonal IgA gammopathy. IEAP responded to dapsone treatment (100 mg/day). Two months after disappearance of active cutaneous lesions, the patient developed generalized lymphadenopathy due to CD30+ anaplastic T-cell lymphoma.
我们报告了一例62岁的患者，其患有皮肤外CD30 +间变性大T细胞淋巴瘤，之前有表皮内IgA脓疱病（IEAP）。临床表现与Sneddon-Wilkinson病相同，组织学检查显示角层下脓疱伴轻度棘层松解。直接免疫荧光仅显示基底上层角质形成细胞的质膜上有IgAK沉积。间接免疫荧光和免疫电泳未显示单克隆IgA丙种球蛋白病的证据。IEAP对氨苯砜治疗（100毫克/天）有反应。活动性皮肤病变消失两个月后，患者因CD30 +间变性T细胞淋巴瘤出现全身淋巴结肿大。
