Case report
Cutaneous forms of mastocytosis in children – report of three cases. Diagnosis and treatment

Mastocytoses consist of heterogenic entities which are characterized by increased mast cell proliferation within skin, more seldom in internal organs and skeletal and haematopoietic systems. Clinical course of the disease depends on the patient’s age, type of mastocytosis, internal organs’ involvement, concomitant diseases as well as on personal response to the applied treatment. Multiple forms of mastocytoses can be distinguished and cutaneous mastocytoses (i.e. urticaria pigmentosa sometimes with blister formation, generalized skin mastocytosis, mastocytoma), of usually slight course, are observed mainly among children, while systemic mastocytoses occur especially in adults. In children spontaneous remission of the disease is often observed; very rarely lesions may persist till adulthood or systemic mastocytosis may develop.
Three cases of children with diagnosed cutaneous mastocytoses are presented: a 14-month and 13-year-old girl and a 3.5-year-old boy. All the presented patients developed infiltrative and blistering skin lesions in the first year of life. Clinical investigation revealed strongly positive Darier’s sign in the girls, while in the boy it was hardly seen. Physical and abdominal ultrasound examination as well as laboratory tests showed no abnormalities; thus there were no clinical indications for bone marrow biopsy. Based on clinical picture and laboratory test results a diagnosis of skin mastocytosis (urticaria pigmentosa) was established in the presented cases. Currently the patients are being treated with oral antihistaminic agents, periodically with topical glucocorticosteroids, and are being followed up in the Outpatient Department of Dermatology in the Medical University of Lodz.