Abstract

Mast cell disease has seldom been associated with hemorrhagic accidents1,2or with coagulation abnormalities such as prolonged coagulation time, prolonged bleeding time, abnormal prothrombin concentration in tolerance test for heparin. Some of the above anomalies have been explained by an associated liver alteration3; others by the presence in plasma of inhibitors of the coagulation process.4-9In most instances, however, the laboratory findings have been minimal or the cases did not show clinical manifestations. Urbach, Bell, and Jacobson10by local friction on cutaneous lesions of urticaria pigmentosa demonstrated a temporary increase of blood heparin in their cases. Berlin11found in blood obtained from a cutaneous lesion an abnormal coagulation process. We have had the opportunity to observe a child with nodular urticaria pigmentosa with severe bleeding from the biopsy site, who required a whole blood transfusion. The coagulation defect was due to the presence of a

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