Urticária pigmentosa: mastocitose cutânea na infância
Urticaria pigmentosa: cutaneous mastocytosis in a child
Claudia Salvini B. M. da Fonseca; Elisângela Ferreira Vasconcellos; Juliana Salvini B. M. da Fonseca; Raquel Machado Barbosa; Vivian Santos C. Oliveira
Resumo
A mastocitose é uma doença rara caracterizada pela proliferação e subsequente acúmulo de mastócitos na pele e/ou órgãos internos. Esta patologia pode ser dividida em cutânea e sistêmica. A cutânea é mais frequente na infância; o infiltrado de mastócitos é limitado à pele, geralmente tem uma evolução benigna e regride espontaneamente na adolescência. A urticária pigmentosa é a apresentação cutânea mais comum. São máculas, pápulas, nódulos ou placas, de coloração vermelho-acastanhada, com predileção pelo tronco. O sinal de Darier ocorre em 90% dos pacientes. Este trabalho tem como objetivo relatar um caso de mastocitose cutânea na infância, no qual o quadro clínico e a biópsia de pele foram suficientes para o diagnóstico. Em pacientes com mastocitose, é importante enfatizar a necessidade do acompanhamento periódico com realização de exames laboratoriais e de imagem, conforme a sintomatologia apresentada pelo paciente, e informar a família sobre a doença e acerca da maior predisposição à anafilaxia.
Palavras-chave
Abstract
Mastocytosis is a rare disease characterized by proliferation and subsequent accumulation of mast cells in the skin and/or internal organs. Presentation can be cutaneous or systemic. The cutaneous form is more common in childhood; mast cell infiltration is limited to the skin, clinical course is usually benign, and the disease regresses spontaneously in adolescence. Urticaria pigmentosa is the most common cutaneous presentation. It includes macules, papules, nodules, or plaques, brownish red in color, with a predilection for the trunk. Darier’s sign is present in 90% of the patients. The present study reports on a case of cutaneous mastocytosis in childhood in which clinical evaluation and skin biopsy were sufficient to establish diagnosis. In patients with mastocytosis, regular monitoring with laboratory tests and imaging exams is extremely important, according to the symptoms presented by the patient. Likewise, the need to inform the family about the characteristics of the disease and the higher risk of anaphylaxis is underscored.
Keywords
Referências
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Submetido em:
20/07/2012
Aceito em:
30/10/2013
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