Department of Dermatology and Allergology, Paracelsus Medical University Salzburg, Salzburg, Austria.
J Dtsch Dermatol Ges. 2021 Apr;19(4):563-581. doi: 10.1111/ddg.14509.
Primary cutaneous lymphomas are extranodal non-Hodgkin lymphomas of T- or B- cell origin, that predominantly affect older patients but have been reported in all age groups and as early as in the first years of life. Diagnosis of cutaneous lymphomas is challenging and requires high clinical suspicion and close collaboration between dermatologists, pediatric oncologists and pathologists. Skin involvement of non-Hodgkin lymphomas in children or adolescents can either be primary cutaneous or secondary due to an underlying nodal lymphoma. The most common primary cutaneous lymphomas encountered in children are of T-cell origin, with mycosis fungoides being the most prevalent cutaneous T-cell lymphoma, followed by CD30 lymphoproliferative disorders. While cutaneous lymphomas share clinicopathologic characteristics between juvenile and adult forms, there are important differences in terms of clinical presentation, diagnosis and treatment. The hypopigmented variant of mycosis fungoides seems to be overrepresented in the pediatric age group. Prognosis and treatment of mycosis fungoides are stage dependent. The majority of children present with early-stage disease and respond well to topical corticosteroids and phototherapy.
原发性皮肤淋巴瘤是 T 细胞或 B 细胞来源的结外非霍奇金淋巴瘤，主要影响老年患者，但也有报告发生于所有年龄组，甚至在生命的最初几年。皮肤淋巴瘤的诊断具有挑战性，需要皮肤科医生、儿科肿瘤学家和病理学家之间高度的临床怀疑和密切合作。儿童的非霍奇金淋巴瘤皮肤受累可以是原发性的，也可以是由于潜在的淋巴结淋巴瘤引起的继发性的。在儿童中最常见的原发性皮肤淋巴瘤是 T 细胞来源的，蕈样真菌病是最常见的皮肤 T 细胞淋巴瘤，其次是 CD30 淋巴增生性疾病。虽然皮肤淋巴瘤在青少年和成人形式之间具有临床病理特征，但在临床表现、诊断和治疗方面存在重要差异。蕈样真菌病的色素减退型似乎在儿科年龄组中更为常见。蕈样真菌病的预后和治疗取决于分期。大多数儿童表现为早期疾病，对局部皮质类固醇和光疗反应良好。
