Gadzia Joseph, Kestenbaum Thelda
Department of Medicine, Division of Dermatology, University of Kansas Medical Center, 3901 Rainbow Boulevard, Kansas City, KS 66160, USA.
J Am Acad Dermatol. 2004 Feb;50(2 Suppl):S4-8. doi: 10.1016/s0190-9622(03)01557-3.
Granulomatous slack skin is a rare cutaneous disorder with less than 50 cases presented in the English-language literature. The disease is characterized by circumscribed erythematous lax skin accentuated most commonly in the axillary and inguinal areas. A strong association with a preceding or evolving diagnosis of mycosis fungoides or Hodgkin's disease has been reported. Previous reports describe the entity as a lymphoproliferative disease in the same spectrum as mycosis fungoides and Hodgkin's disease with a monoclonal T-cell population. Our case, without an evident beta-T-cell receptor rearrangement, suggests that not all cases of granulomatous slack skin are a result of an indolent lymphoma. Granulomatous slack skin probably represents a spectrum of diseases that can eventuate into a lymphoproliferative process.
肉芽肿性皮肤松弛症是一种罕见的皮肤疾病，英文文献报道的病例不足50例。该病的特征是局限性红斑性松弛皮肤，最常见于腋窝和腹股沟区域。据报道，它与蕈样肉芽肿或霍奇金病的先前诊断或病情进展密切相关。既往报道将该疾病描述为与蕈样肉芽肿和霍奇金病同属一个谱系的淋巴增殖性疾病，具有单克隆T细胞群体。我们的病例未发现明显的β-T细胞受体重排，这表明并非所有肉芽肿性皮肤松弛症病例都是惰性淋巴瘤的结果。肉芽肿性皮肤松弛症可能代表了一系列最终可发展为淋巴增殖过程的疾病。
