Lüftl Matthias, Seybold Heidi, Simon Miklós, Burgdorf Walter
Hautklinik, Universitätsklinikum Erlangen, Germany.
J Dtsch Dermatol Ges. 2006 Mar;4(3):236-8. doi: 10.1111/j.1610-0387.2006.05907.x.
Progressive nodular histiocytosis is an extremely rare skin disease is clinically characterized by the coincidence of two distinct lesions, namely, superficial xanthomatous papules up to 5 mm and deep nodules and tumors 1-3 cm. Histologically the nodules represent spindle cell xanthogranulomas. We report a 24-year-old women with these findings. The distinction from other non-Langerhans cell histiocytoses, in particular multiple juvenile xanthogranulomas, which may be more likely to show spontaneous remission, is somewhat unclear; patients with progressive nodular histiocytosis usually follow a serious and disfiguring clinical course.
进行性结节性组织细胞增多症是一种极其罕见的皮肤病，其临床特征是两种不同病变同时存在，即直径达5毫米的浅表黄色瘤丘疹和1至3厘米的深部结节及肿瘤。组织学上，这些结节代表梭形细胞黄色肉芽肿。我们报告了一名有这些表现的24岁女性。与其他非朗格汉斯细胞组织细胞增多症，特别是可能更易出现自发缓解的多发性幼年黄色肉芽肿的鉴别，目前尚不完全明确；进行性结节性组织细胞增多症患者通常会经历严重且毁容的临床病程。
