Department of Medicine, University of Toronto, Toronto, ON, Canada.
Department of Dermatology, Sunnybrook Health Sciences Centre, Toronto, ON, Canada.
J Eur Acad Dermatol Venereol. 2020 Jan;34(1):59-73. doi: 10.1111/jdv.15931. Epub 2019 Oct 14.
Lymphomatoid papulosis (LyP) is a benign chronic often relapsing skin condition that belongs to the CD30-positive cutaneous lymphoproliferative disorders. LyP typically presents as crops of lesions with a tendency to self-resolve, and morphology can range from solitary to agminated or diffuse papules and plaques to nodules or tumours. The clinical-histological spectrum can range from borderline cases to overlap with primary cutaneous anaplastic cell lymphoma (pcALCL). Histology and immunophenotype commonly show overlap with other CD30-positive disorders and sometimes may be identical to pcALCL, making its diagnosis more difficult. Patients with LyP have an increased risk of developing a second neoplasm such as mycosis fungoides, pcALCL and/or Hodgkin lymphoma. Clinical correlation allows its proper classification and diagnosis, which is fundamental for treatment and prognosis. This review focuses on the clinical appearance, histopathological features, diagnosis, differential diagnosis and management of LyP.
蕈样肉芽肿病（LyP）是一种良性的慢性疾病，通常反复发作，属于 CD30 阳性皮肤淋巴增生性疾病。LyP 通常表现为病变的皮疹，有自行消退的趋势，形态可以从单发、成群或弥漫性丘疹和斑块到结节或肿瘤。临床组织学谱可以从边界病例到与原发性皮肤间变性大细胞淋巴瘤（pcALCL）重叠。组织学和免疫表型通常与其他 CD30 阳性疾病重叠，有时可能与 pcALCL 相同，这使得诊断更加困难。LyP 患者发生第二种肿瘤（如蕈样肉芽肿病、pcALCL 和/或霍奇金淋巴瘤）的风险增加。临床相关性允许对其进行适当的分类和诊断，这对治疗和预后至关重要。本文重点介绍 LyP 的临床表现、组织病理学特征、诊断、鉴别诊断和治疗。
