Amyloidosis cutis dyschromica in two siblings and review of the epidemiology, clinical features and management in 48 cases
Amyloidosis cutis dyschromica in two siblings and review of the epidemiology, clinical features and management in 48 cases
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DOI：
10.1111/ajd.12342
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发表时间：
2016-11-01
影响因子：
2
通讯作者：
Agnew, Karen
中科院分区：
文献类型：
--
作者：
Mahon, Caroline;Oliver, Fergus;Agnew, Karen
色素异常性皮肤淀粉样变性（ACD）是一种罕见的原发性皮肤淀粉样变性（PCA）。在皮肤病学文献中缺乏指导其诊断、调查和治疗的信息。我们提出了两个兄弟姐妹与ACD和总结的流行病学，临床特征，自然史和治疗48例ACD的文献。家族性病例比散发病例更常见（37例）。ACD主要报告于东亚和东南亚种族（63%）。家族性病例的平均发病年龄为6岁，散发病例的平均发病年龄为23岁。家族性和散发性ACD的临床特征无显著差异。瘙痒是唯一的症状，占所有病例的19%。未报告系统性淀粉样变性的ACD病例。据报告，阿维A导致10名接受治疗的患者中有7名患者病情改善。不需要进行系统性受累的常规检查。阿维A可能有帮助。
Amyloidosis cutis dyschromica (ACD) is a rare form of primary cutaneous amyloidosis (PCA). There is a paucity of information in the dermatology literature to guide its diagnosis, investigation and treatment. We present two siblings with ACD and summarise the epidemiology, clinical features, natural history and treatments in 48 cases of ACD from the literature. Familial cases were more common (37) than sporadic cases. ACD is predominantly reported in those of East and South-East Asian ethnicity (63%). The mean age of onset was 6 years in familial cases, and 23 years in sporadic cases. The clinical features of familial and sporadic ACD do not differ substantially. Pruritus was the only symptom, and was reported in 19% of all cases. There were no reported ACD cases with systemic amyloidosis. Acitretin was reported to result in improvement in seven of 10 patients treated. Routine investigation for systemic involvement is not necessary. Acitretin may be helpful.