增殖型类天疱疮一例

A rare case of pemphigoid vegetans

患者女,89岁,因腹股沟、外阴、臀间沟皮疹10个月、水疱3周就诊。皮肤科检查:腹股沟、外阴、臀间沟红白色增生性斑块,斑块部位散在绿豆至豌豆大小的糜烂面及水疱,右腋下及右小腿正常皮肤上散在数个类似水疱,部分破溃结痂。臀间沟皮损组织病理检查:棘层增生肥厚,无棘刺松解,部分区域可见表皮下裂隙和水疱,局灶性真皮浅层水肿,伴嗜酸性粒细胞浸润,真皮浅层血管周围可见淋巴细胞为主的浸润。直接免疫荧光:IgG、C3基底膜带线状沉积,IgM真皮簇状小体阳性,IgA阴性。盐裂皮肤间接免疫荧光:IgG、C3沉积在表皮侧。酶联免疫吸附试验检测血清抗体:BP180抗体26.92 U/ml、BP230抗体68.17 U/ml,桥粒芯蛋白1抗体、桥粒芯蛋白3抗体正常。诊断:增殖型类天疱疮。予口服甲泼尼龙,联合外用卤米松软膏及0.03%他克莫司软膏,皮疹逐渐消退。

A 89-year-old female patient presented with skin lesions of the groin,vulva and intergluteal sulcus for 10 months,and blisters for 3 weeks.Skin examination revealed the red-white hyperplastic plaques on the groin,vulva and intergluteal sulcus,on which mung-bean-to pea-sized erosions and blisters scattered,and several similar blisters were scattered on the right axilla and right leg,some of which were broken and covered with crusts.Histopathological examination of the skin lesion on the intergluteal sulcus showed thickened spinous layer without acantholysis,subepidermal fissures and blisters in some areas,focal papillary dermal edema with eosinophil infiltration,and perivascular infiltration composed mainly of lymphocytes in the superficial dermis.Direct immunofluorescence assay showed linear deposition of IgG and C3 at the epidermal basement membrane zone,clustered deposition of IgM in the dermis,but no IgA deposition.Indirect immunofluorescence on salt-split skin revealed that IgG and C3 were deposited on the epidermal side.Enzyme linked immunosorbent assay(ELISA)showed that serum levels of anti-BP180 and anti-BP230 antibodies were 26.92 U/ml and 68.17 U/ml respectively,and those of anti-desmoglein 1(Dsg1)and anti-Dsg3 antibodies were normal.The patient was diagnosed with pemphigoid vegetans.After the treatment with oral methylprednisolone combined with topical halometasone ointment and tacrolimus 0.03%ointment,the skin lesions gradually subsided.