A Case Report of Systemic Mastocytosis

ABSTRACT

A case of systemic mastocytosis in a 6 year old boy was presented. Skin lesions started to appear and spred several days after birth along with severe itching sensation, and intermitent abdominal pain & constipation developed at the age of 5. On physical examination, he was found to be underdeveloped (Height less than 3rd percentile) with normal vital signs. Liver was felt 1 1/2 fb below costal margin, spleen 2 fb. There were many brown to dark brown-colored irregular shaped maculopapulourticarial skin lesions, scattered all over the body, along with multiple thickened areas, and positive Darier's sign was noted. On upper GI series, many honey-combed filling defect were found throughout small intestine. Skin biopsy confirmed the diagnossis showing multiple scattered infilteration of mast cells.