2015
DOI: 10.1016/j.jaci.2015.05.034
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Large maculopapular cutaneous lesions are associated with favorable outcome in childhood-onset mastocytosis

Abstract: Our data show that patients with MPCM-large lesions compared with those with MPCM-small lesions have a more favorable disease course and suggest exploring the size of cutaneous lesions as a prognostic parameter in childhood-onset MPCM.

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Cited by 67 publications
(74 citation statements)
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References 49 publications
(60 reference statements)
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“…However, in some patients skin lesions persist into adulthood (5,24). More recently, two distinct forms of childhood MPCM have been recognized: a variant characterized by monomorphic small-sized lesions, and a second variant defined by polymorphic (often larger) lesions (24,48). Only the monomorphic form is found in adults, suggesting that only this variant persists into adulthood whereas polymorphic lesions usually resolve (24,48).…”
Section: Most Recent Advances In the Classification And Criteria Defimentioning
confidence: 99%
“…However, in some patients skin lesions persist into adulthood (5,24). More recently, two distinct forms of childhood MPCM have been recognized: a variant characterized by monomorphic small-sized lesions, and a second variant defined by polymorphic (often larger) lesions (24,48). Only the monomorphic form is found in adults, suggesting that only this variant persists into adulthood whereas polymorphic lesions usually resolve (24,48).…”
Section: Most Recent Advances In the Classification And Criteria Defimentioning
confidence: 99%
“…However, recent data suggest that childhood patients with monomorphic, small-sized maculopapular skin lesions tend to develop persistent disease, whereas polymorphic skin lesions often disappear before adolescence. 17,18 Adulthood SM is regarded a persistent disease without spontaneous remission. [1][2][3][4][5][6]17,18 In patients who do not develop skin lesions, SM either remains indolent and undetected for many years (occult SM) or the disease is aggressive.…”
Section: Incidence and Prevalencementioning
confidence: 99%
“…17,18 Adulthood SM is regarded a persistent disease without spontaneous remission. [1][2][3][4][5][6]17,18 In patients who do not develop skin lesions, SM either remains indolent and undetected for many years (occult SM) or the disease is aggressive. 1,[4][5][6]12 In fact, patients with ASM or MCL often present without skin lesions.…”
Section: Incidence and Prevalencementioning
confidence: 99%
“…1,2 L a O r g a n i z a c i ó n M u n d i a l d e la Salud (OMS) ha clasificado las mastocitosis de la siguiente manera: (1) mastocitosis cutáneas (MC), en las cuales la infiltración mastocitaria solo ocurre en la piel, y (2) mastocitosis sistémicas (MS), en las cuales existe, al menos, un órgano extracutáneo afectado (Tabla 1). 3,4 El órgano más frecuentemente comprometido es la piel, seguido por la médula ósea y el tracto gastrointestinal. 5 El inicio de las mastocitosis puede ocurrir tanto en la edad pediátrica como en la adultez.…”
Section: Introductionunclassified
“…Estas formas de compromiso cutáneo han sido clasificadas en 3 subtipos: (1) urticaria pigmentosa (UP), también llamada mastocitosis maculopapular; (2) mastocitosis cutánea difusa; (3) mastocitoma solitario. 3,6 Según nuestra consideración, la UP representa un desafío diagnóstico por varias razones: (1) puede ser confundida con una serie de exantemas que se presentan en la infancia y llevar al retardo del diagnóstico; (2) puede generar síntomas sistémicos como consecuencia de la degranulación mastocitaria, que pueden confundirse con otras patologías más comunes en el niño; (3) por la necesidad de dar indicaciones precisas para prevenir Tabla …”
Section: Introductionunclassified