Expression of WT-1 by the vascular component of acral pseudolymphomatous angiokeratoma of children.
Acral pseudolymphomatous angiokeratoma of children (APACHE) is a disease comprised by a dense dermal infiltrate of B-lymphocytes and T-lymphocytes in which prominent blood vessels with plump endothelium are found. In the past, the lesion was interpreted as a variant of angiokeratoma, a vascular malformation, or a nevus. Currently, most authors consider it to be a type of pseudolymphoma with prominent blood vessels. The latter express CD34 and D2-40, while they lack the expression of Glut-1. The expression of Wilms tumor-1 (WT-1) by APACHE has not yet been studied. In this report, we present a case of APACHE on the right foot of a 4-year-old boy and demonstrate immunoexpression of WT-1 by the blood vessels of the lesion. We also performed serial sections and demonstrated that the WT-1+ vessels with prominent endothelium were D2-40-.
Department of Cellular Pathology, Hospital El Bierzo, Ponferrada, Spain.
Angel
A
Fernandez-Flores
Socorro
S
Fierro
Margarita
M
Larralde
D000794:Angiokeratoma / Q000098:blood supply* / Q000378:metabolism* / Q000473:pathology; D001808:Blood Vessels / Q000378:metabolism / Q000473:pathology; D002675:Child, Preschool; D005528:Foot / Q000098:blood supply / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D019310:Pseudolymphoma / Q000378:metabolism* / Q000473:pathology; D012878:Skin Neoplasms / Q000098:blood supply* / Q000378:metabolism* / Q000473:pathology; D025721:WT1 Proteins / Q000097:blood*
D025721:WT1 Proteins; C583751:WT1 protein, human
APACHE; acral angiokerathomatous pseudolymphoma; papular angiolymphoid hyperplasia; pseudolymphomatous angiokeratoma