伴有原发性色素沉着性结节性肾上腺皮质疾病及散在色素沉着的卡尼综合征。

Carney's complex with primary pigmented nodular adrenocortical disease and spotty pigmentations.

作者信息

Ohara N, Komiya I, Yamauchi K, Ohtsuka H, Nagasawa Y, Takeda T, Takasu N

机构信息

Department of Geriatrics, Endocrinology and Metabolism, Shinshu University School of Medicine, Matsumoto, Japan.

出版信息

Intern Med. 1993 Jan;32(1):60-2. doi: 10.2169/internalmedicine.32.60.

DOI:10.2169/internalmedicine.32.60

PMID:8495049

Abstract

Carney's complex is composed of myxoma, spotty pigmentation and endocrine overactivity. A 27-year-old male was diagnosed to have Carney's complex on the basis of intense spotty pigmentations on his face, soles and palms, and bilateral adrenal nodular hyperplasia on computed tomography scanning (CT) and magnetic resonance imaging (MRI). Total bilateral adrenectomy was done; histological findings were compatible with primary pigmented adrenocortical disease (PPNAD). Recently, his sister and one of his brothers were suspected to have Carney's complex with PPNAD. We report the first familial case of Carney's complex with PPNAD and spotty pigmentations in Japan.

摘要

卡尼综合征由黏液瘤、斑点状色素沉着和内分泌功能亢进组成。一名27岁男性因面部、足底和手掌出现明显的斑点状色素沉着，以及计算机断层扫描（CT）和磁共振成像（MRI）显示双侧肾上腺结节性增生，被诊断为卡尼综合征。实施了双侧肾上腺全切术；组织学检查结果与原发性色素沉着性肾上腺皮质疾病（PPNAD）相符。最近，他的姐姐和一个兄弟被怀疑患有伴有PPNAD的卡尼综合征。我们报告了日本首例伴有PPNAD和斑点状色素沉着的卡尼综合征家族病例。