Abstract

DIAZ LLANIO, Pedro Antonio et al. Mastocytosis: urticaria pigmentosa. Rev Ciencias Médicas [online]. 2015, vol.19, n.3, pp. 556-563. ISSN 1561-3194.

Mastocytosis is a rare disease. It is characterized by benign or on rare occasions malignant proliferation of mast cells in tissues. It can produce both systemic and cutaneous changes. In pediatric patients the most common cutaneous form is urticaria pigmentosa with very good prognosis in childhood; usually without systemic manifestations and resolved without sequelae at puberty in 50% of cases. However, it is not well known by doctors as being rare in childhood. The case of a two month-old male patient presented maculo-papulous skin lesions, with brownish symmetrical pink centripetal distribution lesions; scattered and prone to aggregation, presenting a predilection for the trunk and extremities, the diagnosis was: urticaria pigmentosa. The patient has maintained a satisfactory evolution without systemic repercussions or extracutaneous manifestations, he maintains clinical monitoring every six months and annually in dermatology service, in order to observe the natural history of the disease until the age of 5, without cytoreductive therapy; only with symptomatic treatment occasionally.

Keywords : Mastocytosis; Urticaria pigmentosa.

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