Chatterjee Manas, Meru Shweta, Vasudevan Biju, Deb Prabal, Moorchung Nikhil
Department of Dermatology, Command Hospital and AFMC, Pune, India.
Indian J Dermatol. 2012 Nov;57(6):495-7. doi: 10.4103/0019-5154.103074.
A 14-year-old male presented with seven years history of recurrent episodes of fluid filled, itchy and eroded lesions over the body not responding to oral corticosteroids and azathioprine. Dermatological examination revealed crusted plaques and erosions in a seborrheic distribution. Histopathology of skin lesions and direct immunofluorescence were characteristic of pemphigus foliaceus. He was treated with dexamethasone pulse therapy with inadequate response. However, relapsing skin lesions revealed a circinate arrangement with a predilection to trunk and flexures. In view of clinical features suggestive of IgA pemphigus, he was started on dapsone, to which he responded dramatically in four weeks. However, repeat biopsy continued to reveal features of pemphigus foliaceus and ELISA for anti-desmoglein 1 antibodies was positive.
一名14岁男性，有7年病史，全身反复出现充满液体、瘙痒且糜烂的皮损，对口服皮质类固醇和硫唑嘌呤治疗无效。皮肤科检查发现脂溢性分布的结痂斑块和糜烂。皮肤病变的组织病理学和直接免疫荧光检查具有落叶型天疱疮的特征。他接受了地塞米松冲击治疗，但反应不佳。然而，复发的皮肤病变呈环状排列，好发于躯干和屈侧。鉴于临床特征提示IgA天疱疮，开始使用氨苯砜治疗，四周后他对此反应显著。然而，重复活检仍显示落叶型天疱疮的特征，抗桥粒芯糖蛋白1抗体的酶联免疫吸附测定呈阳性。
