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ISSN 1405-1699 (Print)
Órgano Oficial del Colegio Mexicano de Alergia, Asma e Inmunología Pediátrica y de la Asociación Latinoamericana de Pediatría

2015, Number 3

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Alerg Asma Inmunol Pediatr 2015; 24 (3)

Cutaneous mastocytosis: pediatrician should know

Maldonado-Colín G, Campos-Cabrera BL, García-Romero MT

Language: Spanish
References: 24
Page: 78-85
PDF size: 299.42 Kb.


ABSTRACT

Cutaneous mastocytosis (MC) is a heterogeneous group of clinical syndromes characterized by abnormal infiltration of mast cells in different tissues and concomitant release of chemical mediators by these cells. The etiology is unknown; however, mutations have been described in the c-kit proto oncogene and increased colony stimulating factor. Childhood mastocytosis is classified in urticarial pigmentosa (the most common form), solitary mastocytoma, diffuse cutaneous mastocytosis and telangiectasia macularis eruptive perstans. The diagnosis is clinical, where the appearance of the lesions is used to identify the type of MC, Darier´s sign is pathognomonic and positive in 90% of cases, and skin biopsy is the definitive diagnosis. It should be done differential diagnosis with urticaria, angioedema, bullous impetigo, juvenile xanthogranuloma and allergic contact dermatitis. The bone marrow aspirate was considered those patients with clinical evidence of extracutaneous symptoms. Currently, it is not curative, but should be avoidance of triggering factors and use histamine receptor antagonist if the patient has symptoms. Most mastocytosis in children will only MC and tend to resolve spontaneously over time.


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