Barnadas Maria A, Gilaberte Montserrat, Pujol Ramón, Agustí Manuela, Gelpí Carmen, Alomar Augostín
Department of Dermatology, Hospital de la Sta. Creu i St. Pau, Barcelona, Spain.
Int J Dermatol. 2006 Sep;45(9):1089-92. doi: 10.1111/j.1365-4632.2004.02517.x.
A 65-year-old woman had a history of deep vein thrombosis and depression. Psoriasis was diagnosed in 1986 and various topical and systemic therapies, singly or in combination, were prescribed: tar, topical corticosteroids, cyclosporine, etretinate, and methotrexate. Two courses of oral and one course of bath psoralen plus UVA (PUVA) therapy (cumulative dose, 467 J/cm(2)) and UVB (2.96 J/cm(2)) had been given. In January 1999, she developed a flare of generalized psoriasis. In May 1999, therapy with PUVA (8-methoxypsoralen) plus topical acetonide triamcinolone 0.1% was initiated. At the time, she was taking acenocoumarol, lorazepam, and hydroxyzine chlorhydrate. In August 1999, at session 30, when the dose of UVA was 9 J/cm(2), and the total dose was 205 J/cm(2), a bulla appeared on the dorsum of the toe and was controlled with topical antibiotics. Five further sessions of PUVA were given and a generalized itching bullous eruption appeared all over the body. PUVA was stopped and the patient was hospitalized. On physical examination, extensive psoriatic plaques plus vesicles and bullae on the normal skin and on psoriatic lesions were observed all over the body (Fig. 1). Histopathologic study of a lesion showed a subepidermal vesicle containing fibrin, neutrophils, and a few eosinophils. No sunburn cells were observed (Fig. 2). The direct immunofluorescence (DIF) test of perilesional uninvolved skin revealed immunoglobulin G (IgG) (Fig. 3) and C3 at the dermal-epidermal junction. The DIF study using the patient's skin, previously treated with 1 m NaCl, localized the IgG at both the epidermal and dermal sides of the basement membrane zone (Fig. 4). Bullous pemphigoid (BP) was diagnosed and therapy with prednisone (60 mg/day) was started. The disease was well controlled in 3 weeks. The dose of prednisone was tapered and stopped 20 months later, without any recurrence. Study of the antibodies by the indirect immunofluorescence (IIF) test, using monkey esophagus and guinea pig as substrate, was positive at a titer of 1/160 in September 1999. The titer decreased to 1/10 in January 2000, and was negative in July 2000. An enzyme-linked immunosorbent assay (ELISA) test, performed using the commercial kit MBL, which identifies antibodies directed against epitopes of the extracellular fragment NC16 of antigen 2 of BP, was positive at 15 U/mL (normal value, < 9 U/mL) in September 1999, and negative in July 2000 (Table 1).
一名65岁女性有深静脉血栓形成和抑郁症病史。1986年诊断为银屑病,曾单独或联合使用多种局部和全身治疗方法:焦油、局部皮质类固醇、环孢素、依曲替酯和甲氨蝶呤。已进行了两个疗程的口服及一个疗程的浴用补骨脂素加紫外线A(PUVA)治疗(累积剂量,467 J/cm²)以及紫外线B(2.96 J/cm²)治疗。1999年1月,她出现了泛发性银屑病的发作。1999年5月,开始使用PUVA(8-甲氧基补骨脂素)加0.1%局部曲安奈德治疗。当时,她正在服用醋硝香豆素、劳拉西泮和盐酸羟嗪。1999年8月,在第30次治疗时,当紫外线A剂量为9 J/cm²且总剂量为205 J/cm²时,脚趾背出现一个水疱,并用局部抗生素控制。又进行了5次PUVA治疗,随后全身出现泛发性瘙痒性大疱性皮疹。停用PUVA并将患者收住院。体格检查发现全身正常皮肤和银屑病皮损上有广泛的银屑病斑块以及水疱和大疱(图1)。对一处皮损进行组织病理学检查显示表皮下水疱,内含纤维蛋白、中性粒细胞和少量嗜酸性粒细胞。未观察到晒伤细胞(图2)。对皮损周围未受累皮肤进行直接免疫荧光(DIF)检测,在真皮表皮交界处发现免疫球蛋白G(IgG)(图3)和C3。使用先前用1 m NaCl处理过的患者皮肤进行DIF研究,将IgG定位在基底膜带的表皮侧和真皮侧(图4)。诊断为大疱性类天疱疮(BP),并开始用泼尼松(60 mg/天)治疗。3周内病情得到良好控制。泼尼松剂量逐渐减少,20个月后停药,无任何复发。1999年9月,采用间接免疫荧光(IIF)检测法,以猴食管和豚鼠为底物检测抗体,滴度为1/160呈阳性。2000年1月滴度降至1/10,2000年7月为阴性。使用MBL商业试剂盒进行的酶联免疫吸附测定(ELISA)试验,该试剂盒可识别针对BP抗原2细胞外片段NC16表位的抗体,1999年9月为15 U/mL呈阳性(正常值,<9 U/mL),2000年7月为阴性(表1)。