Histiocytosis X presenting as chronic discharging ears.

Malignant histiocytosis was first described in 1939 by Scott and Smith as histiocytic medullary reticulosis(1). We report a case of Histiocytosis X in a 2year-old by presenting with chronic discharging ears, which is a rare manifestation of the disease. A 2-year-old boy was admitted with complaints of chronic ear discharge and fever of 1 month duration. He had recurrent vague pain abdomen and lethargy for almost same period. There was loss of appetite and significant weight loss. There was history of mild abdominal distension during this period. On direct interrogation, there was history of polyuria and polydypsia. There was no history of cough, respiratory distress, rash, bleeding manifestation, or convulsion. On examination, he was malnourished, irritable and pale. Blood pressure and upper segment/lower segment ratios were normal. There was no lymphnode enlargement or rash. There was serous discharge from both ears and prominent abdomen with dilated veins. Liver was enlarged 3 cm below costal margin with a smooth surface and sharp margin and spleen palpable 8 cm, firm in consistency. There was no ascites. There were 5-6 circular punched out lesions over skull which measured 1-1.5 cm in diameter. On the 4th day of admission, petechial rashes appeared over the scalp and gluteal region. Examination of other systems were normal. Investigations showed: hemoglobin7.9 g/dl, TLC-7900/cu mm, N-70%, E2%, ESR 57 mm in first hour, peripheral smear—hypochromic microcytic anemia; VOLUME 32-APRIL1995