Diagnosis and Treatment of Cutaneous Mastocytosis in Children

Abstract

Mastocytosis is a disease with many variants, all of which are characterized by a pathologic increase in mast cells in cutaneous tissue and extracutaneous organs such as the bone marrow, liver, spleen and lymph nodes. The disease presents in two primary age-related patterns: pediatric-onset mastocytosis and adult-onset mastocytosis, which may differ in their clinical manifestations and disease course. Pediatric-onset mastocytosis commonly is diagnosed prior to 2 years of age, and usually consists of cutaneous disease, with urticaria pigmentosa (UP) the most common pattern. The course of pediatric-onset mastocytosis is variable. This is in contrast to adult onset disease which generally presents with systemic findings and increases in extent and severity over time. Because pediatric forms of mastocytosis often differ in presentation and prognosis from adult variants, it is most important to understand pediatric mastocytosis and not rely on adult approaches as a guide on how to identify and manage disease. This is especially important in selecting therapy where antiproliferative agents have a very different set of concerns when used to treat adult mastocytosis compared to pediatric mastocytosis, especially in terms of long-term toxicity. This review is directed at providing age-specific information surrounding the care of the child with mastocytosis.

Anesthetic considerations in pediatric mastocytosis: a review

“…Most mastocytosis cases present in children under the age of 2 years where it is usually confined to the skin and runs a benign, albeit bothersome, course, with resolution in most cases at approximately 9-10 years of age [1,6,13,15]. 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].…”

“…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].…”

“…There may also be a relationship between pediatric mastocytosis and some cases of attention deficit or autism disorder [24]. Cardiovascular shock and hemorrhage are rarely seen in the pediatric mastocytosis population in the absence of extensive skin involvement [1].…”

“…Mastocytosis is characterized by an increase in mast cell activity in the body [1]. First described in the late nineteenth century, mastocytosis is an orphan disease with an estimated overall prevalence of less than 0.4 % of the population [2][3][4][5][6].…”

“…Under a currently recognized WHO-classification scheme, mastocytosis is also subdivided into two general groups, a cutaneous form and a systemic form [12]. Cutaneous mastocytosis subtypes include urticaria pigmentosa (the overwhelming majority of children with mastocytosis), solitary cutaneous mastocytoma (10-35 % of children with skin disease), and diffuse cutaneous mastocytosis (seen almost exclusively in children) [1,[12][13][14]. The more severe or systemic forms of mastocytosis, involving the bone marrow or other extracutaneous sites, are typically seen in the adult population but in only approximately 10 % of children with cutaneous disease [5].…”