Tufted angioma presenting with subclinical coagulopathy

Tufted angioma is a rare, benign vascular tumor of uncertain pathogenesis, characterized histopathologically by "tufts" of capillaries within the dermis. A life-threatening coagulopathy, Kasabach-Merritt phenomenon, occurs in approximately 10% of cases of tufted angioma and is characterized by profound thrombocytopenia and fibrinogen consumption. We present an asymptomatic 10-month-old boy who presented with an erythematous patch of the right upper extremity and subsequently was diagnosed by biopsy with tufted angioma. Baseline laboratory workup of our patient revealed mildly decreased fibrinogen and elevated D-dimer levels without thrombocytopenia or elevated partial thromboplastin time. This suggests that asymptomatic patients with tufted angioma may present with coagulopathy in the absence of Kasabach-Merritt phenomenon. This also highlights the importance of obtaining baseline laboratory workup in patients presenting with tufted angioma.