Rizzo Fernanda Arruda, Vilar Enoi Guedes, Pantaleão Luciana, Fonseca Eliene Carvalho, Magrin Paula Ferrazzi, Henrique-Xavier Marcus, Rochael Mayra Carrijo
Universidade Federal Fluminense/ Hospital Universitário Antônio Pedro, Niterói, Rio de Janeiro, Brazil.
Dermatol Online J. 2012 Mar 15;18(3):5.
Mycosis fungoides (MF), the most common form of cutaneous T cell lymphoma (CTCL), is mainly manifested in the elderly. However, it has been described in children and teenagers.
To report six patients with mycosis fungoides diagnosed in patients under 20 years of age. Our focus is on epidemiologic data, clinical features, histopathological aspects, and immunophenotypical findings.
The files of all patients diagnosed with CTCL at Hospital Universitário Antônio Pedro (HUAP) / Universidade Federal Fluminense (UFF), Niterói, Brazil, from 2007 to 2010 were searched to identify patients under 20 years of age. Slides were reviewed with routine methods (H&E) and immunohistochemical stains by two dermatopathologists and one surgical pathologist.
Among a total of 66 patients with MF, six were children and adolescents between five and nineteen years of age. Most of them had dark skin and presented with the hypopigmented variant of MF; some expressed the T cell CD8+ phenotype, although the prognosis remains the same as for classical CTCL. The main histopathological findings were basilar lymphocytes, Pautrier microabscesses, eccrine infiltration, and dermal fibrosis. One patient had shown pityriasis lichenoides chronica for 10 years before the diagnosis of MF.
The incidence of juvenile mycosis fungoides has increased, corresponding to 9.1 percent of patients diagnosed with MF in our institution in four years. In this sample, 83.3 percent of the patients had the hypopigmented variant and 50 percent of them showed the CD3+/CD8+ T cells phenotype. We emphasize the occurrence of pityriasis lichenoides chronica before the onset of MF in one of our cases. This association, although rare, must be considered in cases of atypical evolution of PLC. The diagnosis of hypopigmented MF should also be considered in patients when hypochromic patches are persistent. We would like to highlight the importance of Pautrier microabscesses, basilar distribution, and eccrine involvement by neoplastic lymphocytes as histopathological diagnostic criteria for this variant of MF.
蕈样肉芽肿（MF）是皮肤T细胞淋巴瘤（CTCL）最常见的形式，主要见于老年人。然而，儿童和青少年中也有相关报道。
报告6例20岁以下诊断为蕈样肉芽肿的患者。我们关注的是流行病学数据、临床特征、组织病理学表现和免疫表型结果。
检索2007年至2010年在巴西尼泰罗伊安东尼奥·佩德罗大学医院（HUAP）/弗卢米嫩塞联邦大学（UFF）诊断为CTCL的所有患者的病历，以确定20岁以下的患者。两名皮肤病理学家和一名外科病理学家用常规方法（苏木精和伊红染色）和免疫组化染色对切片进行复查。
在总共66例MF患者中，6例为5至19岁的儿童和青少年。他们大多皮肤黝黑，表现为MF的色素减退型；一些表现为T细胞CD8 +表型，尽管预后与经典CTCL相同。主要组织病理学表现为基底淋巴细胞、帕托利微脓肿、汗腺浸润和真皮纤维化。1例患者在诊断为MF前有10年慢性苔藓样糠疹病史。
青少年蕈样肉芽肿的发病率有所增加，在我们机构四年中诊断为MF的患者中占9.1%。在这个样本中，83.3%的患者为色素减退型，其中50%表现为CD3 + /CD8 + T细胞表型。我们强调在我们的一个病例中，MF发病前出现了慢性苔藓样糠疹。这种关联虽然罕见，但在PLC非典型演变的病例中必须予以考虑。当色素减退斑持续存在时，也应考虑色素减退型MF的诊断。我们想强调帕托利微脓肿、基底分布以及肿瘤性淋巴细胞累及汗腺作为该型MF组织病理学诊断标准的重要性。
