Infantile hemangiomas (IH) are the most common soft-tissue tumors in childhood, occurring in up to 10% of infants. 1,2 The majority of these benign vascular tumors require no treatment; however, in at least 12% of patients, they can become problematic, and depending on their anatomic location and extent of growth, require clinical evaluation and treatment. 1,3,4 In these cases, oral propranolol has emerged as a first-line treatment. 5 While the efficacy of propranolol as a treatment has been well supported through many studies, substantial variability in administration protocols remains. 2 Current FDA prescribing information for Hemangeol™ and provisional consensus guidelines recommend monitoring heart rate (HR) and blood pressure (BP) after the first and second hours following propranolol initiation and after each dose increase. 1,6,7 However, actual initiation and titration practices differ among providers and
Immunization compliance in the United States is declining, in part due to misinformation and fear surrounding adverse vaccination reactions. Recently, there have been data published in the allergy and immunology literature to show that there may be a relationship between routine vaccinations and induction of symptoms in cutaneous mastocytosis patients; however, this has not yet been explored in the dermatology literature. We sought to uncover the prevalence of vaccine reactions due to mast cell activation within our cohort of maculopapular cutaneous mastocytosis (MPCM) patients in order to contribute to ensuring administration safety, managing familial expectations, and encouraging continued adherence. Our results indicate that while incidence of reaction rates may be higher than the national average, they are mild and families should be counseled to follow recommended immunization schedule guidelines.
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.
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