A Severe and Refractory Case of Anti-p200 Pemphigoid Resulting in Multiple Skin Ulcers and Scar Formation

A Severe and Refractory Case of Anti-p200 Pemphigoid Resulting in Multiple Skin Ulcers and Scar Formation
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DOI:
10.1159/000182268
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发表时间:
2009-01-01
期刊:
影响因子:
3.4
通讯作者:
Shimizu, H.
Shimizu, H.
中科院分区:
医学3区
文献类型:
--
作者:
Goto-Ohguchi, Y.;Nishie, W.;Shimizu, H.

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抗p200类天疱疮是最近描述的自身免疫性起泡皮肤病,其特征在于存在针对未鉴定的200-kDa皮肤自身抗原的自身抗体。大多数以前的病例已经成功地使用轻度至中度的免疫抑制治疗,这导致了良好的预后。我们在此报告一位53岁女性罹患抗p200类天疱疮的严重且难治性病例,其水疱导致多处皮肤溃疡,随后形成严重疤痕。在本病例中,甲基强的松龙冲击治疗足以有效降低疾病活动度。版权所有(C)2008 S. Karger AG,巴塞尔
Anti-p200 pemphigoid is a recently described autoimmune blistering skin disease that is characterized by the presence of autoantibodies against an unidentified 200-kDa dermal autoantigen. Most of the previous cases have been successfully treated using mild-to-moderate immunosuppressive therapies, which resulted in a good prognosis. We report here a severe and refractory case of anti-p200 pemphigoid that developed in a 53-year-old woman, in which blisters led to multiple skin ulcers, followed by severe scar formation. In the present case, methylprednisolone pulse therapy was effective enough to reduce the disease activity. Copyright (C) 2008 S. Karger AG, Basel