Pediatric maculopapular cutaneous mastocytosis: Retrospective review of signs, symptoms, and associated conditions

Emily A. Gurnee, Maija L. Johansen, Thuy L. Phung, Emily L. Guo, Aaron Fong, Megha Tollefson, Henry Nguyen, Heather Brandling-Bennett, Natalie Moriarty, Amy S. Paller, Thy N. Huynh, Leslie P. Lawley*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

2 Scopus citations

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 languageEnglish (US)
Pages (from-to)159-163
Number of pages5
JournalPediatric dermatology
Volume38
Issue number1
DOIs
StatePublished - Jan 1 2021

Keywords

  • maculopapular cutaneous mastocytosis
  • mastocytosis
  • pediatrics
  • tryptase
  • urticaria pigmentosa

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Dermatology

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