Skin Cancer Center, Department of Dermatology, Allergology and Phlebology, Bremerhaven Reinkenheide Medical Center, Bremerhaven, Germany.
Dermatopathology Laboratory, Lübeck, Germany.
J Dtsch Dermatol Ges. 2020 Mar;18(3):199-205. doi: 10.1111/ddg.14041. Epub 2020 Feb 26.
Lymphomatoid papulosis (LyP) is characterized by a varied clinical presentation that includes erythema, papules, pustules, vesicles, plaques, nodules and ulcerations. While its biological course is typically marked by spontaneous regression, the histopathological findings of LyP are consistent with cutaneous T-cell lymphoma. Provided patients do no develop a secondary lymphoma, they exhibit unusually high 10-year survival rates (> 90 %), which is a typical feature of LyP. To date, the etiology and pathogenesis of LyP have not been elucidated. One particular subtype of LyP is known to be associated with chromosome 6p25.3 rearrangement (DUSP22-IRF4 translocation). Treatment is guided by the clinical presentation. In addition to a wait-and-see approach, recommended options include topical corticosteroids and PUVA therapy.
蕈样肉芽肿（LyP）的临床表现多样，包括红斑、丘疹、脓疱、水疱、斑块、结节和溃疡。尽管其生物学过程通常以自发性消退为特征，但 LyP 的组织病理学表现与皮肤 T 细胞淋巴瘤一致。只要患者不发展为继发性淋巴瘤，他们的 10 年生存率就会异常高（>90%），这是 LyP 的一个典型特征。迄今为止，LyP 的病因和发病机制尚未阐明。有一种特定类型的 LyP 与染色体 6p25.3 重排（DUSP22-IRF4 易位）有关。治疗方案取决于临床表现。除了等待观察外，推荐的治疗方法还包括局部皮质类固醇和 PUVA 治疗。
