Mastocitosis cutánea: reporte de un caso / Cutaneous mastocytosis: A case report

Introducción La mastocitosis representa un grupo de enfermedades caracterizadas por una acumulación excesiva de mastocitos en uno o múltiples tejidos. Puede limitarse a la piel o tener un compromiso sistémico, siendo de baja prevalencia y pronóstico benigno en la infancia. Objetivo Reportar un caso de urticaria pigmentosa como subtipo de mastocitosis cutánea y hacer una revisión bibliográfica enfocada en los hallazgos clínicos, el diagnóstico y el manejo inicial básico. Caso clínico Lactante de 6 meses de edad con múltiples máculas y pápulas de color café claro localizadas en el tronco, los brazos y las piernas, cuadro compatible con una urticaria pigmentosa, confirmada mediante biopsia. Se solicitaron exámenes para descartar compromiso sistémico. La paciente fue tratada con medidas generales, educación y antihistamínicos, con excelente evolución. Conclusiones La mastocitosis cutánea es una enfermedad poco común, de buen pronóstico. En la infancia generalmente bastan las medidas generales y educación para obtener resultados favorables. La terapia farmacológica de primera línea son los antagonistas H1.

Introduction Mastocytosis represents a group of diseases characterised by an excesive accumulation of mastocytes in one or multiple tissues. It can affect only the skin, or have a systemic involvement. It has a low prevalence, and the prognosis is benign in children. Objective To report a case of urticaria pigmentosa as a subtype of cutaneous mastocytosis, and present a literature review focused on clinical findings, diagnosis and initial basic management. Clinical case A child of six months of age presenting with multiple blemishes and light brown papules located on the trunk, arms and legs. The symptoms were compatible with urticaria pigmentosa, and was confirmed by biopsy. Tests to rule out systemic involvement were requested. The patient was treated with general measures, education, and antihistamines, with favourable results. Conclusions Cutaneous mastocytosis is a rare disease with a good prognosis. In childhood general measures and education are usually enough to obtain favourable results. Histamine H1 antagonists are the first line drug treatment.

Urticária pigmentosa em lactentes: relato de um caso e revisão da literatura / Urticaria pigmentosa in infants: a case report and revision of literature

Paciente pediátrico, 6 meses, masculino, internado no Hospital Geral em Caxias do Sul. Apresentou lesões hipercrômicas maculopapulares descamativas, vermelho-acastanhadas, intensamente pruriginosas, atingindo grande parte do corpo. Recebeu anti-histamínicos e banhos de água e aveia, enquanto internado. Foi realizada biópsia de pele, que constatou quadro compatível com urticária pigmentosa (UP). A UP é a mais freqüente manifestação de mastocitose, ocorrendo na pele e caracterizando-se por lesões cutâneas com pigmentação persistente e pruriginosa. A mastocitose é caracterizada por um acúmulo local ou sistêmico de mastócitos, sendo que os sintomas, dentre os quais o principal é o prurido, resultam da liberação maciça de histamina e outros mediadores pelos mastócitos. O diagnóstico é baseado basicamente na história e apresentação clínica, com confirmação de envolvimento cutâneo por biópsia. Ainda não há tratamento definitivo e eficaz, e somente alívio sintomático pode ser atingido (AU)

Pediatric patient, 6 months old, male, admitted to General Hospital in Caxias do Sul. The patient displays hyperchromic maculopapular peeling lesions, reddish-brown, with intense itching extending over the majority of the body. The patient received anti-histamines and oat water baths while at the hospital. The results of a skin biopsy were indicative of urticaria pigmentosa (UP). UP is the most common manifestation of mastocytosis, occurring in the skin and characterized by cutaneous lesions with persistent and itching spots. Mastocytosis is characterized by a local or systemic accumulation of mast cells, accompanied by symptoms, among which the primary one is itching, resulting from the massive release of histamine and other mediators by the mast cells. Diagnosis is based basically on clinical history and examination, confirmed by cutaneous biopsy. There is still no satisfactory and efficient treatment, and only the symptoms can be alleviated (AU)

Panorama actual de la mastocitosis / Actual panorama of the mastocytosis

La mastocitosis cutánea es un padecimiento relativamente frecuente en el paciente pediátrico y poco conocido por el médico no dermatolólogo. Se revisan su fisiopatología, clínica y terapéutica, haciendo énfasis en la posibilidad de afección extracutánea.