Majeed H A, Quabazard Z, Hijazi Z, Farwana S, Harshani F
Department of Pediatrics, Faculty of Medicine, University of Kuwait.
Q J Med. 1990 Jun;75(278):607-16.
In a six-year study of 46 children with familial Mediterranean fever (recurrent hereditary polyserositis), 20 children (43 per cent) developed cutaneous manifestations. Ten children had 18 episodes of erysipelas-like erythema which proved to be specific for the disease. Twelve children (26 per cent) had 31 episodes of non-specific purpuric rash and six children (13 per cent) had nine episodes of Henoch-Schönlein purpura. Other manifestations included diffuse erythema of the face, and/or trunk, angioneurotic oedema, diffuse erythema of the palms and soles followed by mild desquamation of the skin, Raynaud's phenomenon and a subcutaneous nodule. The mean frequency of the cutaneous lesions was 1.6/patient/year before colchicine therapy and 0.4/patient/year after colchicine therapy (p = 0.0005). The high incidence of cutaneous manifestations and their response to colchicine strongly suggest that skin involvement is an integral part familial Mediterranean fever.
在一项针对46名患有家族性地中海热（复发性遗传性多浆膜炎）儿童的为期六年的研究中，20名儿童（43%）出现了皮肤表现。10名儿童发生了18次丹毒样红斑发作，这被证明是该疾病的特异性表现。12名儿童（26%）出现了31次非特异性紫癜性皮疹发作，6名儿童（13%）出现了9次过敏性紫癜发作。其他表现包括面部和/或躯干弥漫性红斑、血管神经性水肿、手掌和脚底弥漫性红斑，随后皮肤轻度脱屑、雷诺现象和一个皮下结节。在秋水仙碱治疗前，皮肤病变的平均发作频率为1.6次/患者/年，秋水仙碱治疗后为0.4次/患者/年（p = 0.0005）。皮肤表现的高发生率及其对秋水仙碱的反应强烈表明，皮肤受累是家族性地中海热的一个组成部分。
