Nodular and bullous cutaneous mastocytosis of the xanthelasmoid type: casereport

Severe generalized nodular and bullous mastocytosis of the xanthelasmoid ty pe is described in a 7-month-old boy. Reddish to yellowish-brown xanthelasm oid papules and nodules first developed in the inguinal region a few weeks after birth and then progressively spread to cover nearly the entire body s urface. There was severe pruritus and recurrent episodes of blistering. The diagnosis of cutaneous mastocytosis of the xanthelasmoid type with subepid ermal bullae was confirmed by skin biopsies showing solid and deeply penetr ating infiltrates of metachromatic mast cells under light and electron micr oscopy. Systemic involvement of other organs, however, was excluded by bone scintigraphy, abdominal ultrasound, bone marrow aspiration and echocardiog raphy. The extensive skin involvement was reflected in highly elevated urin ary levels of histamine (263.4 mug L-1) and its metabolite N-methylimidazol e acetic acid (20.8 mg L-1). The patient was systematically well and receiv ed only symptomatic treatment. Over a period of 1 year, the condition gradu ally improved, and the skin lesions began to flatten and regress.