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

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.

Cutaneous mastocytosis in childhood

“…Blistering was reported in a third of the children, flushing in 25%, gastrointestinal symptoms (abdominal pain, diarrhea, nausea) in 20%, and anaphylaxis in 5%. In other cohorts, similar, but sometimes also lower, symptom rates were observed [ 13 , 18 ]. In particular, the occurrence of anaphylactic reactions in pediatric CM varies between 0 and 9% in the literature [ 13 , 19 ].…”

Pediatric Cutaneous Mastocytosis: A Case Report and Literature Review

“…It is a disorder that has a wide range of symptoms and therefore a broad differential diagnosis including epidermolysis bullosa, café-au-lait macules, linear immunoglobulin A bullous dermatosis, atopic dermatitis, and angioedema must be considered. 5,6 The most recent WHO classification of mastocytosis states that to diagnose pediatric cutaneous mastocytosis, there must be the typical skin lesions, a positive Darier's sign, the absence of clinical signs of systemic involvement. 7 Bone marrow biopsy is not necessary.…”

“…It is a disorder that has a wide range of symptoms and therefore a broad differential diagnosis including epidermolysis bullosa, café-au-lait macules, linear immunoglobulin A bullous dermatosis, atopic dermatitis, and angioedema must be considered. 5,6…”

Mast Cell Activation Disorders

“…Maculopapular CM (MPCM), also known as urticaria pigmentosa, with its two variants, monomorphic and polymorphic [32]. Clinically, it is recognizable for its small, monomorphic, red-brown macules/papules ,typically on the trunk, sparing face and extremities [33].…”

“…In most cases, CM develops with a benign course and often regresses by puberty. Recent data showed that localized mastocytomas tend to regress at a rate of 10% per year (Gurnee), while MPCM presents a slower rate of 1.9% per year [32,36,37].…”