Dermatología Pediatrica

Postępy Dermatologii i Alergologii  1/2010

Mastocytoses
Post Dermatol Alergol 2010; XXVII, 1: 50-56

autorzy: Aleksandra Lesiak, Irmina Olejniczak, Joanna Narbutt,


Mastocytoses consist of heterogenic entities which are characterized y increased mast cell proliferation within skin, more seldom in nternal organs and skeletal and haematopoietic systems. Clinical ourse of the disease depends on the patient's age, type of astocytosis, internal organs' involvement, concomitant diseases as ell as on personal response to the applied treatment. Multiple forms f mastocytoses can be distinguished and cutaneous mastocytoses (i.e. rticaria pigmentosa sometimes with blister formation, generalized kin mastocytosis, mastocytoma), of usually slight course, are bserved mainly among children, while systemic mastocytoses occur specially in adults. In children spontaneous remission of the disease s often observed; very rarely lesions may persist till adulthood or systemic mastocytosis may develop.

Three cases of children with diagnosed cutaneous mastocytoses are resented: a 14-month and 13-year-old girl and a 3.5-year-old boy. All he presented patients developed infiltrative and blistering skin esions in the first year of life. Clinical investigation revealed trongly positive Darier's sign in the girls, while in the boy it was ardly seen. Physical and abdominal ultrasound examination as well as aboratory tests showed no abnormalities; thus there were no clinical ndications for bone marrow biopsy. Based on clinical picture and aboratory test results a diagnosis of skin mastocytosis (urticaria igmentosa) was established in the presented cases. Currently the atients are being treated with oral antihistaminic agents, eriodically with topical glucocorticosteroids, and are being followed p in the Outpatient Department of Dermatology in the Medical niversity of Lodz.