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.
Original language | English (US) |
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Pages (from-to) | 159-163 |
Number of pages | 5 |
Journal | Pediatric dermatology |
Volume | 38 |
Issue number | 1 |
DOIs | |
State | Published - Jan 1 2021 |
Funding
This study was conducted in collaboration with the Pediatric Dermatology Research Alliance (PeDRA). Research reported in this publication was supported in part by the biostatistics and bioinformatics of Winship Cancer Institute of Emory University and NIH/NCI under award number P30CA138292. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health.
Keywords
- maculopapular cutaneous mastocytosis
- mastocytosis
- pediatrics
- tryptase
- urticaria pigmentosa
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health
- Dermatology