肉芽肿性皮肤松弛症。组织病理学诊断先于临床表现12年。
Granulomatous slack skin. Histopathology diagnosis preceding clinical manifestations by 12 years.
作者信息
Goldsztajn Karen O, Moritz Trope Beatriz, Ribeiro Lenzi Maria Elisa, Cuzzi Tullia, Ramos-E-Silva Marcia
机构信息
Sector of Dermatology and Post Graduation Course - HUCFF-UFRJ and School of Medicine, Federal University of Rio de Janeiro, Brazil.
出版信息
J Dermatol Case Rep. 2012 Dec 31;6(4):108-12. doi: 10.3315/jdcr.2012.1117.
BACKGROUND
Granulomatous slack skin is a very rare subtype of T-cell cutaneous lymphoma, characterized by the slow development of cutaneous sagging, especially on flexural areas. Its behavior is indolent and the treatment, in the majority of cases, disappointing.
MAIN OBSERVATION
We report a 54-year-old black patient with granulomatous slack skin, who at the beginning of the investigation showed intense xeroderma and generalized lymph node enlargement. The diagnosis was established based on histopathologic findings long before the disease's characteristic clinical presentation appeared.
CONCLUSIONS
During the twelve years of follow-up, the clinical manifestation evolved to marked skin looseness, most predominant in flexural regions, illustrating the clinical hallmark of granulomatous slack skin, long after first histological abnormalities were observed.
背景
肉芽肿性皮肤松弛症是一种非常罕见的T细胞皮肤淋巴瘤亚型，其特征是皮肤松弛发展缓慢，尤其是在屈侧部位。其病程呈惰性，在大多数情况下，治疗效果令人失望。
主要观察结果
我们报告了一名54岁的肉芽肿性皮肤松弛症黑人患者，在调查开始时，患者表现为严重的皮肤干燥和全身淋巴结肿大。早在该病典型的临床表现出现之前，就根据组织病理学检查结果确诊了。
结论
在12年的随访中，临床表现发展为明显的皮肤松弛，最主要出现在屈侧部位，这说明了在首次观察到组织学异常很长时间后，肉芽肿性皮肤松弛症的临床特征。
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