Multifocal Kaposiform Hemangioendothelioma Successfully Treated With Sirolimus Monotherapy

Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor that typically presents in infancy and may be associated with the Kasabach–Merritt phenomenon (KMP). We present a challenging case of multifocal KHE on the leg of an infant, initially suspected at birth to be a reticulate port wine birthmark. Skin biopsy and imaging supported the rare diagnosis of multifocal KHE. Complicated by KMP, he was started on sirolimus monotherapy with significant improvement in his widespread disease.