Tufted Angioma Associated with Kasabach-Merritt Phenomenon: A Therapeutic Challenge

Multimodal treatment of Kasabach-Merritt syndrome arising from tufted angioma: A case report

“…The term TA was introduced by Wilson-Jones and Orkin in 1976, based on the characteristic histology of the lesion (8). TA is a solitary tumor or infiltrated plaque believed to have more of an inflammatory appearance than a vascular abnormality (5). The lesion has been described as small, cannonball-like, circumscribed angiomatous tufts and nodules in the dermis and subcutaneous tissue with characteristic lymphangioma-like vessels (9).…”

“…Numerous therapeutic modalities have been employed for KMS, with no clear evidence that any type of treatment is superior over others. It has been suggested that multidisciplinary treatment is required for KMS (5). Several treatment regimens for KMS have been reported, including topical or systemic corticosteroid, IFN, chemotherapy, radiation, laser, propranolol, sirolimus and surgery (5,11-19; Table I).…”

Reddish-brown plaque on the left buttock

“…About 15% of cases have a congenital occurrence and may appear at birth but about 50% occur during the 1 st year of life and a great majority appear under 10 years of age. Although in most report, TA involves both sexes equally, [1][2][3][4] but in the largest series of childhood tufted angioma to date by Osio et al, [2] male sex was clearly predominant (9 of 13 patients/69%). [2] Acquired TA has also been reported in adults.…”

“…[5] The patients with TA may occasionally undergo complications with a serious even life threatening platelet trapping Kasabach Merritt syndrome (KMS) which is a thrombocytopenic coagulopathy due to sequestration of platelet and coagulation factors within vessels of tumor. [1,8,9] The frequency of KMS in TA is uncertain, but regarding the evolution of TA it may be advisable to obtain at least a complete blood cell count. Importantly, platelet count of less than 1,50,000/µl should prompt a more extensive evaluation for coagulopathy.…”

“…Treatment options include: compression therapy, high dose of systemic corticosteroids, vincristine, embolization, surgical removal (choice for noncongenital small lesions), IFN α (in cases that are refractory to systemic corticosteroids), cryosurgery, pulse dye laser, chemotherapy or radiotherapy. [1,6,7,10] Potent topical steroids have been used for reduction of pain. Recurrence of TA after surgery have been described.…”