沙利度胺联合小剂量激素治疗结节性类天疱疮1例报告

Combination Therapy of Thalidomide and Low-dose Corticosteroid in the Treatment of Pemphigoid Nodularis

患者,男性,64岁,全身反复红斑、丘疹、结节2年伴重度瘙痒。体检:头、颈、躯干及四肢对称性泛发暗红色丘疹、斑块、结节,多数结节顶端见粟米至绿豆大小的糜烂或凹陷性结痂,未见明显水疱。直接免疫荧光示C_3沿表皮基底膜带线状沉积;间接免疫荧光示循环基底膜C_3滴度1:80;组织病理示表皮下水疱;诊断为结节性类天疱疮,予小剂量糖皮质激素联合沙利度胺治疗,取得较满意疗效。

We report a 64 -year old male patient with recurrent generalized erythema, papule and nodularis -like eruption with severe pruritus for 2 years. Generalized, symmetrical distribu- tion of dark red papules, plaques and hard nodules were found on head, neck, trunk and limbs. Erosion or pitting scars with various size between green bean and corn were seen on the surface of most of nodules. No obvious blister was observed. Direct immunofluorescence （DIF） showed the deposition of C3 in a linear pattern at the basement membrane zone. Indirect immunofluorescence （IIF） disclosed circulating anti -basement membrane zone C3 with titre of 1：80. Histopathology showed blisters in subepidermal. The diagnosis was pemphigoid nodularis on the basis on the investigation of clinical features. Combination therapy of thalidomide and low - dose corticosteroid was administrated with satisfactory responses.