以色素沉着为首发表现的POEMS综合征1例报告

Report of a case of POEMS syndrome first presented with pigmentation

目的总结原发性肾上腺皮质功能减退的诊断思路及POEMS综合征的内分泌改变的临床特点。方法对1例"乏力、色素沉着3年"为主诉的原发性肾上腺皮质功能减退患者进行临床分析及病因探讨,复习文献并总结临床特点。结果患者以乏力、消瘦、皮肤色素沉着为主要表现;化验提示贫血,单克隆免疫球蛋白,VEGF升高;合并脾大、淋巴结肿大;存在亚临床肾上腺皮质功能减退,肾上腺结构未见异常;合并多发周围神经病变;淋巴结活检符合浆细胞型Castleman病。最终确诊为POEMS综合征。本例患者存在亚临床肾上腺皮质功能减退,为POEMS综合征的表现之一。结论原发性肾上腺皮质功能减退应进一步查找其病因。内分泌改变是POEMS综合征常见的临床表现。色素沉着可能为系统性疾病(如POEMS综合征)的皮肤改变,应进一步挖掘其病因。Castleman病是POEMS综合征的常见病因,淋巴结结构可有特征性改变,确诊需完整淋巴结的病理。

Objective To understand the diagnosis process of primary adrenal insufficiency and the endocrinopathy of POEMS syndrome. Methods We presented one case of primary adrenal insufficiency with chief complaint of ＂fatigue and pigmentation for 3 years＂. The literatures was reviewed and the clinical characteristics were summarized. Results The main manifestations of the patients were fatigue, pigmentation and weight loss.Laboratory results suggested anemia, monoclonal immunoglobulin and increased VEGF level.The patient was diagnosed subclinical adrenal insufficiency, butadrenal glands appeared structurally normal. Combined with multiple peripheral neuropathy. Lymph node biopsy conformed the diagnostic of Castleman disease.The final diagnosis was POEMS syndrome. Adrenal insufficiency was one of the endocrine changes of POEMS syndrome. Conclusion The etiology of primary adrenal insufficiency should be identified in all patients with confirmed disease. Endocrinopathy is a central feature of POEMS syndrome, including hypogonadism, hypothyroidism, adrenal insufficiency et al. Pigmentation may be a feature of systemic disease（such as POEMS syndrome）. Castleman disease is a common cause of POEMS syndrome. Biopsy of lymph node can confirm the diagnosis.