Congenital Violaceous Plaque in a Toddler

A 19-month-old boy presented with a painless, violaceous, keratotic plaque on the left leg, with an erythematous border and overlying coarse telangiectasias. The lesion was present since birth, and grew in diameter and volume commensurate with growth of the patient to 1 x 3 cm. There were several occasions of bleeding from trivial trauma followed by hemorrhagic crusting. Histological examination shows verrucous epidermal hyperplasia with numerous dilated vessels abutting the epidermis, many of which contain blood, serum, or both. The vascular component involves predominantly the superficial to mid dermis, with the largest vessels present in the papillary dermis. As the vascular diameter and density decreases with descent into the deep dermis, there are an increased number of eccrine glands. Clinicopathologic features demonstrated a combination of eccrine angiomatous hamartoma and verrucous venous malformation, rendering a diagnosis of eccrine angiokeratomatous hamartoma. Eccrine angiomatous hamartoma appears at birth or early childhood as a benign solitary nodule or plaque on the lower extremities. Histologic features include mid to deep dermal eccrine gland hyperplasia and interspersed thin-walled capillaries, admixed with pilar, lipomatous, mucinous, and lymphatic tissue. Verrucous venous malformation is a congenital vascular anomaly that presents as solitary or multiple hyperkeratotic nodules on the lower extremities. Histologic features include verrucous epidermal hyperplasia and an increased number of thin-walled vessels in the superficial and deep dermis and sometimes subcutaneous tissue. EAKH represents a unifying diagnosis to account for the presence of combined features of eccrine angiomatous hamartoma and verrucous venous malformation.