Current approach to cutaneous mastocytosis in childhood

Abstract

Mastocytosis is a heterogeneous disorder characterized by clonal proliferation and accumulation of mast cells in one of more organs which may lead to different clinical pictures. Pathological increase and activation of mast cells in various tissues can cause different clinical pictures. Cutaneous mastocytosis limited to the skin is the most typical clinical picture observed in children and systemic mastocytosis is very rare in the pediatric age group. The diagnosis of cutaneous mastocytosis is based on clinical findings, but is often delayed due to lack of clinical awareness of the disease and lack of its consideration in the differential diagnosis. This article focuses on the current diagnosis, management and treatment of cutaneous mastocytosis in children in order to increase awareness about this issue.(Turk Pediatri Ars 2016; 51: 123-7)

Cutaneous Mastocytosis in Childhood—Update from the Literature

“…Trigger factors, such as cold water, hot baths, or exercise, may cause the lesions to blush [ 27 ]. Additionally, pediatric patients sometimes associate with an atopic pathology [ 28 , 29 ].…”

Effective management of severe cutaneous mastocytosis in young children with omalizumab (Xolair® )

“…Omalizumab (Xolair ® ; Genentech, San Francisco, CA, USA) 150 mg was administered subcutaneously monthly for 3 months. A modified weekly Mastocytosis Symptom Assessment Form (MSAF) (Fig. a,b), completed prior to each injection revealed a decreased score with treatment.…”

“…Mastocytosis is characterized by excessive accumulation of mast cells in tissues. Whereas cutaneous mastocytosis (CM) is limited to the skin, systemic mastocytosis (SM) involves extracutaneous organs . Various triggers can lead to mast cell degranulation and give rise to problems such as urticaria, bullae formation, pruritus and/or gastrointestinal problems.…”

“…Whereas cutaneous mastocytosis (CM) is limited to the skin, systemic mastocytosis (SM) involves extracutaneous organs. 1 Various triggers can lead to mast cell degranulation and give rise to problems such as urticaria, bullae formation, pruritus and/or gastrointestinal problems. Rarely, massive mediator release can cause lifethreatening systemic presentations such as anaphylaxis.…”

Diffuse Cutaneous Mastocytosis in a Child - a Case Report

“…In pediatric patients, mastocytosis is usually cutaneous and transient, while in adults the condition commonly progresses to a systemic form. Mastocytosis is a heterogeneous disorder characterized by clonal proliferation and accumulation of mast cells in one or more organs which may lead to different clinical pictures (1). Pathological accumulation of mast cells may affect the skin, bone marrow, liver, spleen and the lymph nodes.…”