Mastocytosis is a heterogeneous group of myeloproliferative disorders characterized by accumulation of clonal mast cells in various tissues. The aim of this study was to determine the symptoms evolution and outcome after 10 years observation.Fifty-five children with mastocytosis were included in the study group and monitored concerning mast cell mediator-related symptoms (MC MRSs) and clinical course of the disease for a period of ≥10 years.Patients presented with a maculopapular cutaneous form of mastocytosis (MPCM) (n = 47) and diffuse cutaneous mastocytosis (DCM) (n = 8). The complete remission (CR) of skin lesions occurred in 10.3% of children after 10 years observation; no remission (NR) was observed in 17.9% children. The CR of skin specific MC MRS occurred in 69.2% children with MPCM and in 14.3% with DCM.Most children with cutaneous mastocytosis (CM) eventually experience a major or partial regression of skin lesions, although complete regression before puberty is rare. The spontaneous remission of skin specific MC MRS is less frequent in children with DCM.
Justyna Czarny, Joanna Renke, Anton Żawrocki, Roman J Nowicki, Magdalena Lange