[伴有原发性肾上腺皮质功能不全的POEMS综合征]
[POEMS syndrome with primary adrenocortical insufficiency].
作者信息
Mauvais-Jarvis F, Bertherat J, Ravaud H, Thomopoulos P, Luton J P
机构信息
Clinique des Maladies endocriniennes et métaboliques, Hôpital Cochin, Paris.
出版信息
Presse Med. 1998 Jan 10;27(1):15-7.
BACKGROUND
POEMS syndrome is a multisystem disorder. It usually presents as severe polyneuropathy and monoclonal gammapathy associated with endocrinopathies, organomegaly, skin hyperpigmentation.
CASE REPORT
A patient with POEMS syndrome developed primary adrenocortical deficiency revelated by asthenia and hyperpigmentation. Hydrocortisone replacement therapy produced a rapidly beneficial effect on asthenia and hyperpigmentation.
DISCUSSION
Although asthenia and skin pigmentation are common in POEMS syndrome, adrenocortical deficiency is seldom reported. This endocrinopathy might be underestimated in POEMS patients. We suggest systematic screening with a rapid ACTH test in all POEMS patients in order to detect underlying adrenal deficiency.
背景
POEMS综合征是一种多系统疾病。它通常表现为严重的多发性神经病和单克隆丙种球蛋白病，并伴有内分泌病、器官肿大、皮肤色素沉着。
病例报告
一名POEMS综合征患者出现原发性肾上腺皮质功能减退，表现为乏力和色素沉着。氢化可的松替代疗法对乏力和色素沉着产生了迅速的有益效果。
讨论
虽然乏力和皮肤色素沉着在POEMS综合征中很常见，但肾上腺皮质功能减退很少被报道。这种内分泌病在POEMS患者中可能被低估。我们建议对所有POEMS患者进行快速促肾上腺皮质激素（ACTH）试验的系统筛查，以检测潜在的肾上腺功能减退。
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