Features of the treatment of cutaneous mastocytosis in children

Mastocytosis is a heterogeneous group of diseases with accumulation of clonal mast cells in tissues. Mastocytosis is characterized by complex pathogenesis, diverse clinical picture and ambiguous prognosis. Complex molecular mechanisms, multiorgan damage and variable clinical course require interdisciplinary approaches to diagnostics and development of treatment strategy for mastocytosis. In patients with symptoms associated with mast cell mediators, strict avoidance of any triggers leading to activation and degranulation of mast cells is of great importance. Treatment of cutaneous mastocytosis in children does not have standard recommendations and should consider the clinical form and type of cutaneous mastocytosis, severity of mediator symptoms, age, concomitant and comorbid diseases. Etiotropic drug therapy for cutaneous mastocytosis has not yet been developed, therefore, in pediatric practice, agents for symptomatic treatment of the disease are used. The article systematizes the data of international experience in the management of children with cutaneous and systemic mediator symptoms of varying severity. The characteristics (mechanism of action, indications) of groups of antimediator drugs most often used in outpatient pediatric practice are given. Emphasis is placed on the need to correct the level of vitamin D in children with mastocytosis. Data on the perioperative management of children with cutaneous mastocytosis are analyzed. Skin care and external therapy are relevant for clinical forms of the disease, accompanied by the formation of weals, bullae and itching. Observation of children should be carried out by specialists-dermatovenerologists and allergists-immunologists, with experience in working with this pathology. Children with cutaneous mastocytosis may require psychological or psychiatric help. Families of patients should be informed about the clinical features, the need for dynamic observation, the relevance of preventive and symptomatic therapy, and the prognosis of the disease. Timely prescribed anti-mediator therapy will prevent the development of severe mediator symptoms, improve the quality of life, and improve the prognosis in children with cutaneous mastocytosis.

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