J Korean Pediatr Soc.
1979 Jul;22(7):546-549.
A Case Report of Systemic Mastocytosis
- Affiliations
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- 1Department of pediatrics, Kyung Pook National University, School of Medicine, Taegu, Korea.
- 2Deprtment of Pathology, Fatima hospital, Taegu, Korea.
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.