Kassabach-Merritt Syndrome:
Kassabach-Merrit (KM) syndrome is a consumption coagulopathy that occurs as
a complication of angiomatous nevi. Angiomatous nevi leading to KM syndrome
are generally single, deep lesions of large size, but the disorder can vary
occasionally and occur in association with smaller and more superficial lesions.
The responsible angiomas most commonly occur on the trunk, neck and proximal
parts of the limbs, particularly the thighs and shoulders, and are relatively
uncommon on the head. This picture is of an 2 month old infant who was brought
to the pediatric outpatient department for evaluation of a swollen left arm
since birth. On examination the limb was enveloped by a rapidly growing hemangioma.
Although the child appeared well and the hemangioma was not symptomatic, a small
ulcer was noted near the elbow, and the platelet count was less than 10,000.
