Raffin D, Delaplace M, Roussel A, Estève E
Service de dermatologie, hôpital Porte-Madeleine, CHR d'Orléans, 1, rue Porte-Madeleine, 45032 Orléans cedex 1, France.
Ann Dermatol Venereol. 2013 Dec;140(12):784-7. doi: 10.1016/j.annder.2013.07.005. Epub 2013 Aug 13.
Anti-p200 pemphigoid is a recently described autoimmune subepidermal bullous dermatosis characterized by its target antigen and the associated anatomoclinical picture. The treatment is not as yet well defined.
A 73-year-old man consulted for a pruritic bullous eruption with buccal involvement. Direct immunofluorescence revealed linear deposits of IgG and C3 at the dermal-epidermal junction. Elisa screening for circulating anti-BP180 and anti-BP230 antibodies was negative. A diagnosis of bullous pemphigoid was suspected. After an unfavourable clinical outcome under clobetasol and then prednisolone and methotrexate, other immunological tests were performed. Indirect immunofluorescence on NaCl-cleaved skin revealed a deposit of IgG4 antibodies on the dermal side. Immunoblotting showed antibodies directed against a 200-kDa antigen on a dermal extract. A diagnosis of anti-p200 pemphigoid was made. The patient was treated with dapsone combined with prednisolone. Seventy-two hours later, treatment was stopped due to hepatic cytolysis related to immunoallergic hepatitis. Treatment with mycophenolate mofetil was then initiated and resulted in complete remission, which persisted at seven months.
The diagnosis of anti-p200 pemphigoid was made on the basis of a set of clinical and immunological factors. Anti-p200 pemphigoid differs from standard bullous pemphigoid in terms of more frequent cephalic, acral and mucous membrane involvement, as well as a greater degree of miliary scarring. There was no eosinophilia. Elisa screening for anti-BP180 and anti-BP230 antibodies was negative. Immunoblotting showed antibodies directed against a 200kDa protein on dermal extract. The treatment is not well defined, even if dapsone appears to be the most effective therapy. To our knowledge, our patient is the first to be successfully treated with mycophenolate mofetil.
Treatment of anti-p200 pemphigoid is difficult. In our case, treatment by mycophenolate mofetil was effective and could offer an alternative to dapsone.
抗p200类天疱疮是一种最近被描述的自身免疫性表皮下大疱性皮肤病，其特征在于其靶抗原以及相关的解剖临床症状。其治疗方法尚未明确界定。
一名73岁男性因瘙痒性大疱性皮疹伴口腔受累前来就诊。直接免疫荧光显示在真皮 - 表皮交界处有IgG和C3的线性沉积。酶联免疫吸附测定（ELISA）筛查循环抗BP180和抗BP230抗体均为阴性。怀疑为大疱性类天疱疮。在使用氯倍他索，随后使用泼尼松龙和甲氨蝶呤治疗后临床效果不佳，遂进行了其他免疫学检查。对经氯化钠处理的皮肤进行间接免疫荧光检查显示在真皮侧有IgG4抗体沉积。免疫印迹显示在真皮提取物上有针对200 kDa抗原的抗体。诊断为抗p200类天疱疮。患者接受了氨苯砜联合泼尼松龙治疗。72小时后，由于免疫过敏性肝炎导致肝细胞溶解而停止治疗。随后开始使用霉酚酸酯治疗，结果完全缓解，这种缓解状态持续了7个月。
抗p200类天疱疮的诊断基于一系列临床和免疫学因素。抗p200类天疱疮与标准大疱性类天疱疮不同，其头面部、肢端和黏膜受累更为频繁，以及粟丘疹瘢痕形成程度更高。无嗜酸性粒细胞增多。ELISA筛查抗BP180和抗BP230抗体均为阴性。免疫印迹显示在真皮提取物上有针对200 kDa蛋白的抗体。治疗方法尚未明确界定，尽管氨苯砜似乎是最有效的治疗方法。据我们所知，我们的患者是首例成功接受霉酚酸酯治疗的病例。
抗p200类天疱疮的治疗较为困难。在我们的病例中霉酚酸酯治疗有效，可为氨苯砜治疗提供一种替代方案。
