When to Suspect Systemic Mastocytosis?

Background : Mastocytosis is a rare disease in children and it usually resolves by adolesence.However; it causes anxiety in parents and should be taken into consideration in patients with cutaneous eritematous lesions.

Aim: To find out the systemic mastocytosis patients who apply to pediatric hematology , pediatric allergy and dermatology policlinics.

Material and Methods: The files of 24 patients diagnosed with mastocytosis between January 2018 and August 2018 in our clinic were retrospectively analyzed.

Results: All patients had cutaneous mastocytosis, 14 patients had urticaria pigmentosa and 9 patients had solitary mastocytoma and one patient had diffuse cutaneous mastocytosis. The male-female ratio was: 2/1. The median age for onset of disease was 7,2 (0-48 months )months. The patients had different types of skin findings such as macules (n=16), plaques(n=6), papules(n=4) and bullae(n=3). None of the patients had anaphylaxis but two patients had angioedema. The diagnosis was confirmed with skin biopsy in 8 patients. The other were diagnosed due to typical history and physical examination.

None of the patients had cytopenia, hepatosplenomegaly.Only one patient triptase level >20 ug/l. The bone marrow aspiration and biopsy was done to 3 patients and all were normal.The patients were treated with H2 antihistaminics, local moisturizing creams and topical corticosteroid drugs in case of undesirable pruritus.

Conclusions: Most cases of childhood mastocytosis are observed in the form of cutaneous mastocytosis and ıt usually resolves by adolescence. If the patients have cytopenia, hepatosplenomagaly or triptase level >20 ug/l the bone marrow aspiration and biopsy is recommended.