Acral pseudolymphomatous angiokeratoma of children: a case report with immunohistochemical study of antipodoplanin antigen.

Acral pseudolymphomatous angiokeratoma of children (APACHE) is characterized by multiple angiomatous papules on the hands and feet in children. Here, we report a case of APACHE in a female patient followed up from 13 to 19 years of age with a dark red lesion on the center of the dorsum of the right thigh. Histologically, vacuolar alteration and exocytosis of lymphocytes, and specific dense cellular infiltration beneath the epidermis to the reticular dermis were found. On immunolabeling study, the lesion vessels were found to be positive for both the lymphatic endothelium-specific marker podoplanin and blood vessel-specific marker CD34. These findings suggested that APACHE is a type of vascular malformation.