Presentation of cutaneous mastocytosis in 173 children

Abstract

This is a retrospective review of the case files and clinical photographs of 173 children diagnosed with cutaneous mastocytosis by a dermatologist in an exclusively paediatric practice. Of the 98 males and 75 females, 51% had mastocytomas, 47% had urticaria pigmentosa and three patients had diffuse cutaneous mastocytosis. Of these cases, 87% first appeared prior to or at 6 months of age. Flushing occurred in 26% of urticaria pigmentosa cases, 29% of mastocytomas and 100% of diffuse cutaneous mastocytosis. Blistering was noted in 23% of urticaria pigmentosa, 31% of mastocytomas and 100% of diffuse cases. Neither symptom was noted in 59% of urticaria pigmentosa and 49% of mastocytomas. There were three cases with a positive family history. The finding of a palmar mastocytoma has only once been previously reported. Illustrated descriptions of our cases are provided.

Cutaneous Mastocytosis in Children: Retrospective Analysis of 41 Patients

“…11,12 Kutanöz mastositozlarda deri lezyonları hastaların çoğunda puberte ile tam veya kısmi remisyon göstermekte-dir. 11 Sistemik mastositoz sıklığı çocuk hastalarda çok azdır.…”

“…Yüzden fazla çocuk hastanın dâhil edildiği bazı çalışmalarda bir ya da iki sistemik mastositoz saptanmıştır. 11,12 Mastositoz lezyonlarının ortaya çıkış yaşının hastalık seyri üzerinde prognostik etkisi bulunmaktadır. 10 Genel olarak mastositozların yaklaşık %15'inde hastalık konjenital, yine yaklaşık %50'sinde lezyonlar hayatın ilk iki yılı içinde ortaya çıkmaktadır.…”

Urticaria pigmentosa: aspectos clínicos y terapéuticos para el pediatra

“…La resolución completa puede ocurrir en un 10% de los pacientes, mientras que una mejoría espontánea importante se observa hasta en un 70% de los pacientes al inicio de la pubertad. 23,39,40 Los datos de casos persistentes son limitados y varían entre un 25% y un 57% de los pacientes .41,42 En general, las lesiones que se presentan después de los 10 años tienden a persistir y a mantenerse sintomáticas.…”

Anesthetic considerations in pediatric mastocytosis: a review

“…Over one-half of children with mastocytosis are symptomatic, and the most common symptoms include flushing and pruritis, mostly related to mastocyte release of histamine, prostaglandin-D2 (PG-2), and leukotriene-C4 [1,4,[16][17][18][19]. Bronchospasm, a typical sequela of IgE-mediated reactions, it not a common mediator-related incident in mastocytosis [17,20,21].…”

“…Fewer than one-half of adolescent cases progress into adulthood, where the course may become systemic in up to 30 % of affected children, usually as an indolent form [13,15,17,19]. It is unclear which disease subtype seen in adolescence will progress into the adult form of the disease [25].…”