中华皮肤科杂志 ›› 2012, Vol. 45 ›› Issue (10): 693-696.

• 论著 • 上一篇    下一篇

H综合征

马东来1,刘佳玮2,方凯3   

  1. 1. 中国医学科学院、北京协和医学院北京协和医院皮肤科
    2. 中国医学科学院、北京协和医学院北京协和医院
    3.
  • 收稿日期:2012-05-08 修回日期:2012-05-28 出版日期:2012-10-15 发布日期:2012-09-29
  • 通讯作者: 马东来 E-mail:mdonglai@public3.bta.net.cn

The H syndrome

  • Received:2012-05-08 Revised:2012-05-28 Online:2012-10-15 Published:2012-09-29
  • Contact: Dong-Lai MA E-mail:mdonglai@public3.bta.net.cn

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摘要：

报道1例H综合征。患者男，18岁。因躯干、四肢进行性皮肤硬化斑伴表面色素沉着和多毛8年就诊。此外，患者还有身材矮小、听力减退、心脏病变、肝脾肿大和阴囊肿块等临床表现。实验室检查结果显示性腺功能减退。皮损的组织病理检查显示表皮棘层肥厚，基底层色素增加。真皮和皮下脂肪组织广泛的纤维化，并有组织细胞、浆细胞和血管周围的淋巴细胞浸润。符合H综合征的诊断。临床上，本病需与Winchester综合征和POEMS综合征等鉴别。

关键词: POEMS综合征

Abstract:

A case of the H syndrome was reported. An 18-year-old man presented with an 8-year history of progressive cutaneous sclerosis, skin hyperpigmentation, and hypertrichosis on the trunk and extremities. In addition, he suffered from short stature, hearing loss, cardiac anomaly, hepatosplenomegaly, and scrotal masses. Laboratory examination revealed hypogonadism. Cutaneous histopathologic examination showed hyperpigmentation of the basal layer with acanthosis, widespread fibrosis （moderate in dermis and severe in subcutis） and an infiltrate composed of histiocytes and plasma cells. A perivascular lymphocyte infiltrate was also observed throughout the dermis and subcutaneous fat tissue. These findings were consistent with the H syndrome. Differential diagnosis must be made with Winchester syndrome and polyneuropathy, organomegaly, endocrinopathy, M-protein and skin change （POEMS） syndrome.

Key words: POEMS syndrome