疱疹样天疱疮17例分析

Clinical analysis of 17 patients with pemphigus herpetiformis

目的探讨疱疹样天疱疮的临床特点、治疗及预后。方法对本院1998-2009年收治的17例疱疹样天疱疮住院患者的临床资料作回顾性分析。结果本病组病例临床以泛发性环状红斑、紧张性水疱为特点,尼氏征阳性仅4(23.53%)例,口腔黏膜受累2(11.76%)例,均伴有剧烈瘙痒。皮损组织病理示:表皮棘层内水疱及嗜酸性粒细胞海绵形成,直接免疫荧光示:棘细胞间IgG(或)和C3沉积。8例予氨苯砜100mg/d治疗,3例7天内有效的控制了病情,另5例无效者加用强的松25～50mg/d,10天内也有效的控制了病情;9例予甲基强的松龙20～40mg/d治疗,3天内4例也有效的控制了病情;另5例无效者,分别予激素加量,或加用雷公藤多甙、硫唑嘌呤、环磷酰胺或丙种球蛋白治疗,14天内4例有效的控制病情,其中1例无效者,予血液透析及血液灌流7次,27天内也有效的控制了病情。结论疱疹样疱疮是有特殊的临床表现及病理特点的一类天疱疮,多数病例单用氨苯砜或加用中小剂量糖皮质激素可控制病情。

Objective To investigate clinical features,treatment and prognosis of pemphigus herpetiformis.Methods The clinical data of 17 patients with pemphigus herpetiformis who were hospitalized from 1998 to 2009 was collected for retrospective analysis.Results Clinical manifestations were specialited for the generalized erythema annular and tensive blister with intense itching.The positive Nikolsky′s sign was only seen in 4 cases （23.53%）,2 cases had oral mucosal involvement （11.76%）.Histopathology showed blister within stratum spinosum epidermidis with eosinophilic sponginess.Direct immunofluorescence showed intercellular IgG and（or） C3 deposition.Treatment：Among 17 patients,8 cases were treated with dapsone 100mg/d,3 cases got better in 7days,and the other 5 cases were also controlled well in 10 days by adding prednisone 25～50mg/d.9 cases were treated with methylprednisolone 20～40mg/d,4 cases were controlled in 3days,another 5 cases who were poor response with corticosteroids were treated with Glucosidorum Tripterygll Totorum,azathioprine,cyclophosphamide,IVIG,4 cases were controlled in 14days,and 1 cases who had no response yet then be hemoperfusion hemodialysis 7 times,and could be controlled within 27days.Conclusion Pemphigus herpetiformis is a variant of pemphigus with specific clinical and pathological features,Most cases can be controlled by DDS or small/middle doses of corticosteroids.