以生殖器糜烂溃疡为首发症状的大疱性类天疱疮一例并文献复习

Bullous pemphigoid with erosions and ulceras on genital as the first symptom: a case report and literature review

大疱性类天疱疮为常见的表皮下自身免疫性疱病,多见于70岁以上老年人,靶抗原主要为定位于基底膜的BP180和BP230,以躯干及四肢屈侧常见,约10%~30%的患者出现黏膜病变,口腔黏膜最常受累。本文报道一例以生殖器糜烂溃疡为首发症状的大疱性类天疱疮患者,男,60岁,阴茎、龟头、冠状沟糜烂、溃疡4个月,胸部反复出现一米粒大小水疱1个月,尼氏征阴性。皮损组织病理示:表皮下陈旧性水疱,真皮浅层淋巴细胞、嗜酸性粒细胞浸润。直接免疫荧光(DIF):表皮基底膜C3阳性,线状沉积,IgG、IgM、IgA阴性。外周血抗BP180、230、Ⅶ型胶原抗体均阴性。诊断:大疱性类天疱疮。给予口服醋酸泼尼松、雷公藤及烟酰胺治疗,半月后生殖器糜烂、胸部水疱均愈合,2个半月后生殖器溃疡愈合。泼尼松逐渐减量,半年后停药,随访2年未复发。

Bullous pemphigoid is the most frequent autoimmune bullous disease and mainly affects elderly individuals.The target antigens are mainly BP180 and BP230 located in the basement membrane,the lesions are common on the trunk and the flexion side of the limbs.About 10%-30%of patients have mucosal lesions,and the oral mucosa is the most commonly involved.Herein,we report a case of bullous pemphigoid with genital erosive ulcer as the first symptom.A 60-year-old male patient presented with a 4-month history of recurrent erosions and ulceras on his penile,and repeated rice-sized blisters of on the chest for 1 month,and.Nikolsky’s sign was negative.The histopathological examination revealed old blister under the epidermis and lymphocytes,eosinophils infiltration in the superficial dermis.Direct immunofluorescence showed linear deposition of C3 along the basal membrane band.The anti-BP180 antibodies,anti-BP230 antibodies and anti-type VII collagen antibodies were negative.The diagnosis of bullous pemphigoid was made.The patient was treated with prednisone acetate,centella and nicotinamidethe,genital erosions and chest blister healed half a month later,glans ulcers healed completely after 2.5 months,the drug was stopped half a year later,and there was no recurrence during 2 years follow-up.