Mastocytosis: retrospective analysis of maculopapular cutaneous form

Abstract

Introduction. The article presents the data of a retrospective analysis of the debut and clinical manifestations of the types of maculopapular cutaneous mastocytosis (МPCM) in 96 children, and the frequency of non-regression of skin rashes with age in 33 adults.Aim. To conduct a retrospective analysis of the onset, clinical manifestations and dynamics of maculopapular cutaneous mastocytosis (MPCM).Materials and methods. Outpatient records of 96 children and 33 adults, who were treated and monitored at the Moscow Scientific and Practical Center of Dermatovenereology and Cosmetology, were statistically analyzed.Results. Monomorphic type of MPCM was diagnosed in 28 (29.2%) patients, polymorphic – in 68 (70.8%) patients. In 92.6% of children with a polymorphic type and in 35.7% with a monomorphic type, the rash debuted at the age of two years. The clinical picture of the monomorphic type was dominated by multiple small brown or yellow-brown spots. Rashes in the polymorphic type were spread, represented by brown or reddish-brown spots, papules, plaques ranging in size from several millimeters to several centimeters. The positive phenomenon of Unna-Darier was determined in 82.3% of cases. In patients with polymorphic type, the general population level of tryptase (<11.4 μg/l) was determined significantly more often than in patients with monomorphic type (p < 0.05). Elevated tryptase level was found in 53.6% of children and 69.7% of adults with monomorphic rashes. Pruritus was the most common cutaneous symptom, occurring in 49.0% of children. Bullous eruptions developed only in patients with polymorphic type. The most frequently reported systemic manifestations were gastrointestinal symptoms and headaches.Conclusions. The obtained results suggest that patients with early onset of the disease with polymorphic and variable lesions show a more favorable outcome.