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Revista chilena de pediatría

Print version ISSN 0370-4106

Abstract

ZEGPI-TRUEBA, María Soledad; HASBUN-ACUNA, Paula  and  BERROETA-MAURIZIANO, Daniela. Cutaneous mastocytosis: A case report. Rev. chil. pediatr. [online]. 2016, vol.87, n.3, pp.204-207. ISSN 0370-4106.  http://dx.doi.org/10.1016/j.rchipe.2015.09.004.

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.

Keywords : Mastocytosis; Urticaria pigmentosa; Mastocytoma; Histamine H1 antagonist.

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