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Pediatric maculopapular cutaneous mastocytosis: Retrospective review of signs, symptoms, and associated conditions
Author(s) -
Gurnee Emily A.,
Johansen Maija L.,
Phung Thuy L.,
Guo Emily L.,
Fong Aaron,
Tollefson Megha,
Nguyen Henry,
BrandlingBennett Heather,
Moriarty Natalie,
Paller Amy S.,
Huynh Thy N.,
Lawley Leslie P.
Publication year - 2020
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/pde.14399
Subject(s) - cutaneous mastocytosis , medicine , systemic mastocytosis , tryptase , dermatology , retrospective cohort study , maculopapular rash , immunology , mast cell , disease , rash
Abstract Background/Objectives Though maculopapular cutaneous mastocytosis is the most common form of pediatric mastocytosis, it remains unclear which patients will experience severe symptoms. We sought to better define the presentation and the cutaneous and systemic signs and symptoms in patients with maculopapular cutaneous mastocytosis. Methods We analyzed retrospective data on 227 patients diagnosed with maculopapular cutaneous mastocytosis prior to age 15 years from five US clinical sites. We collected data on signs, symptoms, age of onset, and laboratory testing. Results Median age of onset of maculopapular cutaneous mastocytosis was 3 months, with 94% of patients presenting prior to age 2 (range 0‐15 years). Patients presenting before age 2 had significantly lower serum tryptase level ( P = .019). Greater number of skin lesions ( P = .006), number of reported skin signs and symptoms ( P < .001), and higher tryptase levels ( P < .001) were associated with more systemic symptoms. Conclusion Children with maculopapular cutaneous mastocytosis, who have greater skin involvement, higher serum tryptase level, and more skin signs and symptoms, are more likely to have systemic symptoms.