Micose fungóide hipopigmentar com 20 anos de evolução

Abstract

Abstract:Hypopigmented mycosis fungoides is a rare variant of the cutaneous T-cell lymphoma, in which hypopigmented lesions are seen without the classical lesions. Only three cases are found in the Latin-American literature. We report the case of a 29-year-old black male patient, who first presented with these lesions 20 years ago. The treatment is similar to the classical forms of the disease: psoralen plus ultraviolet A (PUVA) and topical nitrogen mustard are most often used. Keywords: Phototherapy; Hypopigmentation; Mycosis fungoides. The typical form of this disease's presentation consists of three patterns: a premycotic stage with non-specific erythematous and scaling lesions, which recalls psoriasis and parapsoriasis. The second stage is characterized by the infiltration of pre-existing erythematous-squamous plaques, emergence of new infiltrated plaques and nodules. A third stage is identified by the appearance of tumors of various sizes with an erythematous light-brown color, although initial tumor forms are rare. ResumoIn the pure hypopigmentary variant there do not occur any classic mycosis fungoides lesions. 1A 29 year-old male patient presented with extensive hypochromic spots since nine year of age. The spots were slightly scaling in the bilateral gluteal region. In the region close to the thighs, they were not pruriginous. Similar lesions on the right arm improved after being exposed to the sun.The dermatological examination showed hypochromic spots on the buttocks and in the region lesions merely described as a hypochromic variant. 7 Ardigó et al. demonstrated in their study that, of the seven patients assessed with HMF, five presented with CD4 lymphocyte infiltrate. In addition, they reported previously described cases in which the predominant phenotype was also CD4, contradicting some authors who have described a CD8 infiltrate as typical in these cases. 7 Histologically, atrophy is absent. Moderate-tosevere exocytosis, tending toward weak epidermotropism, is a rare characteristic in premature MF. 3The clinical response in various studies has proved to be faster than in classical MF, irrespective of the therapy utilized. Recurrences have been frequent after periods of one to three years without maintenance therapy, but respond after reinitiating therapy. 5In the literature, various therapeutic options are described, such as ultraviolet radiation as a monotherapy, occlusive corticoid, electron bath, bischloronitrosourea (BCNU). But the most described and best assessed modalities were phototherapy with UVA and UVB, and topical nitrogen mustard.Immunohistochemical findings manifested a higher frequency of CD8 cells in juvenile onset mycosis fungoides and in dark-skinned patients. In older light-skinned patients, CD4 lymphocyte infiltrates are frequent. 1,8 In 1999, Berti, while studying primary cutaneous lymphocytes with an aggressive behavior, identified CD8 as a bad prognosis factor due to dissemination to the central nervous system, lungs, testicles and oral mucosa. 9The T CD8 cell finding has b...

A Variante Hipocrómica da Micose Fungoide no Quotidiano Médico

“…1. Revisão da literatura sobre a micose fungoide O linfoma cutâneo de células T (LCCT) caracteriza-se como um grupo de linfomas com manifestação primária na pele, 4,9 entre os quais a MF é o mais frequente. Acredita-se que o LCCT pode iniciar-se através de estímulo antigénico crónico.…”

“…5,7 A MF é indolente, raramente progride para formas avançadas. 4,5 O tratamento consiste na redução do número das lesões e do alívio dos sintomas associados, como o prurido, estando a melhor opção terapêutica relacionada ao estádio evolutivo da doença. 5,6,14 Máculas e placas são responsivos a terapias direcionadas à pele, a exemplo da quimioterapia tópica à base de mostarda nitrogenada, dos esteroides tópicos e da fototerapia com associação de psoraleno e radiação ultravioleta A (PUVA), ocorrendo, em alguns casos, cura da doença.…”

“…A variante hipocrómica da micose fungoide (MFH) é um linfoma não Hodgkin cutâneo de células T, raro, crónico e indolente, associado a bom prognóstico, com predileção por homens jovens e de raça negra. [1][2][3][4] É frequente o encaminhamento de casos de MFH para serviços especializados pela dificuldade do diagnóstico nos cuidados primários, provocando atraso no tratamento dos pacientes. Atribui-se a dificuldade diagnóstica à patologia não demonstrar sinais alarmantes, ser infrequente, inespecífica ao estudo histopatológico em estádios iniciais, e de se assemelhar a dermatoses comuns, como a dermatite de contato, a psoríase e a hanseníase.…”