氨苯砜综合征诊治探讨——附6例报告

Diagnosis and treatment of dapsone syndrome——a report of six cases.

目的：探讨氨苯砜综合征的诊治要点：方法：对6例氨苯砜综合征患者的临床资料进行回顾性分析。结果和结论：6例氨苯砜综合征患者均为中青年，从服药到出现症状的时间较长，为9～55日.6例患者均以高热起病，起病急骤、凶险，病初均有麻疹紫癜样皮疹，皮疹多形，严重者出现尼氏征阳性或阴性的大疱和（或）全身性红皮病样皮疹；均有浅表淋巴结肿大；多数患者有血液系统损害；并均有肝损害：确诊后及时停氨苯砜，给予足量肾上腺皮质激素和（或）大剂量免疫球蛋白静脉注射，以及支持对症处理，住院12～80（中位数53）日，4例痊愈，1例病情得到控制，另1例因治疗第2日自动出院放弃治疗，死于肝功能衰竭？提示氨苯砜综合征需要及时、正确处理，否则预后不良.

Objective： To discuss the key points for the diagnosis and treatment of dapsone syndrome. Methods： Six cases with dapsone syndrome were retrospectively analyzed. Results and Conclusion： Six cases with dapsone syndrome were all middle-aged and young. Dapsone syndrome had long latent period （9 -55 days）. The onset of dapsone syndrome was acute and severe, manifested with hyperpyrexia. Various rashes were found, appeared as measlesurpura, bullae with or without positive Nikolsky＇s sign and/or erythrodermia. All had superficial lymph nodes enlargement. Most dapsone syndrome had involvement of hematological system. All had liver involvement. The condition would be controlled bv immediate discontinuation of the dapsone combining the use of corticosteroid and/or high-dose intravenous immunoglobulin after definite diagnosis. Supporting and symptomatic treatments were needed. The length of hospitalization was 53 （ 12 -80） days. Four cases recovered completely and were discharged. One case was controlled. One case died of liver function failure after leaving hospital automatically in the second day of treatment. The study suggests that dapsone syndrome should be treated promptly and correctly. Otherwise it would have poor prognosis.