RESUMO
Se presenta el caso de un lactante menor con tumoración cutánea benigna localizada en el aspecto submandibular izquierdo de la cabeza compatible con mastocitoma cutáneo solitario. Esta rara lesión tumoral es parte del grupo heterogéneo de las mastocitosis, conjunto de enfermedades que resulta de la infiltración anómala de mastocitos en diferentes tejidos corporales, afectando particularmente la piel.La variedad más común de mastocitosis en niños es la urticaria pigmentosa, seguida del mastocitoma cutáneo que contabiliza entre el 10 y 15% de todos los casos. Suele situarse en tronco o extremidades, aunque como en este paciente puede localizarse en cabeza uotros sitios corporales. Es de aparición precoz durante la primera infancia o alguna vez congénito; por lo regular no hay participación de órganos internos o situaciones potencialmente letales. Su curso es crónico con remisiones parciales y exacerbaciones sintomáticasmolestas como fenómenos de urticación y desarrollo de ampollas frente a estímulos externos o internos que preocupan a los familiares cercanos. Su reconocimiento clínico precoz y la pronta instauración terapéutica con esteroides locales, antihistamínicos orales u otras medidas pertinentes, aceleran el tiempo de resolución casi siempre espontánea hacia los 2 años de vida; es importante la debida explicación a los padres
This is a report the case of an infant with benign cutaneous tumor located in the left submandibular aspect of the head support solitary cutaneous mastocytoma. This rare tumor lesion is part of the heterogeneous group of mastocytosis, group of diseases resulting from abnormal infiltration of mast cells in various body tissues, particularly affecting the skin. The most common variety of mastocytosis inchildren is urticaria pigmentosa, followed by cutaneous mastocytoma counted between 10 and 15% of all cases. Usually located on the trunk or extremities, although as in this patient may be located in the head or other body sites. Is early onset in infancy or congenital ever, usually no involvement of internal organs or life-threatening situations. Its course is chronic symptomatic partial remissions and exacerbations Urtication annoying as phenomena and development of blisters against external or internal stimuli which trouble close relatives. Its early clinical recognition and prompt establishment local steroid therapy, oral antihistamines or other relevant measures speed time to spontaneous resolution usually 2 years of life, is important due explanation to parents.
Assuntos
Humanos , Masculino , Recém-Nascido , Mastocitoma/patologia , PediatriaRESUMO
Solitary mastocytoma is an infrecuent benign tumor from a group of cutaneous mastocytosis. Common onset in childhood, sometimes congenital. It might be one or few lesions on trunk or limbs, with or whithout general symptoms. Its is self-limited and tends to disappear with time, there is not an effective treatment available.
Assuntos
Mastocitoma Cutâneo , Mastocitose Cutânea , Humanos , LactenteRESUMO
The term urticaria pigmentosa (UP) denotes a heterogeneous group of disorders characterized by abnormal growth and accumulation of mast cells (MC) in the skin. Symptoms result from MC chemical mediator's release, pathologic infiltration of neoplastic MC in tissues or both. Multiple molecular, genetic and chromosomal defects seem contribute to an autonomous growth, but somatic c-kit D816V mutation is more frequently found, especially in systemic disease. The aim of this paper is to provide a current overview for a better understanding of the symptoms associated with this disease, to describe its classification, recent advances in its pathophysiology and its treatment.
