Background: Mastocytosis is a heterogenous disorder characterized by accumulation of mast cells in different tissues. Mastocytosis may be limited to skin or it may involve bone marrow, liver, spleen and lymphatic tissue as systemic mastocytosis. Patients may also present with anaphylaxis. Here we report a six-year-old boy with atopic dermatitis, wheat anaphylaxis, and solitary mastocytoma.

Case report: The dermatologic manifestations of atopic dermatitis appeared early in his first days of life and were all over his face with a dark red infiltrate, edema, small vesicles and severe itching. At the age of 3 months, the patient was fed with an ordinary milk formula for the first time, which immediately resulted in severe vomiting, generalized urticaria and angioedema of the lips and eyes, followed by lethargy and atony. The result of RAST test indicated allergy to wheat and cow’s milk. At the age of two years, his mother noticed a rubber-like skin lesion on his left arm with positive Darier sign. Laboratory tests were all within normal limits except IgE and serum tryptase which were 1425 IU/mL and 31 ng/ml, respectively.

Conclusion: To conclude, Anaphylaxis may accompany cutaneous forms of mastocytosis even mastocytoma. Delayed diagnosis due to the lack of awareness may lead to recurrent life-threatening events, particularly in infancy when anaphylactic reactions are easily misdiagnosed as infants are not capable of expressing their symptoms.