Papular angiolymphoid hyperplasia and lymphoplasmacytic plaque: a clinical and histological spectrum

Abstract

Acral pseudolymphomatous angiokeratoma of children (APACHE) is a rare form of cutaneouspseudolymphoma characterized byangiomatous papules with a predilection for the acral regions of children. Classically, a densedermal lymphocytic infiltrate composed of both T and B cells is seen in histological specimens,together with prominent vessels lined by plump endothelial cells. Increasing evidence suggests thatthis condition is neither necessarily acral, pseudolymphomatous, nor angiokeratomatous. It may notalways be a pediatric disease. Therefore, the correctness of its nomenclature has been questioned.Herein, we report threecases whose clinical and histological features were consistent with the diagnosis of APACHE. To ourknowledge, this is the first report of APACHE from Southeast Asia. We also discuss why we believe"APACHE" to be a misnomer and support "papular angiolymphoid hyperplasia" as a more accurate andencompassing term. In addition, we illustrate a case with significant overlapping features withlymphoplasmacytic plaque in children, suggesting that both entities may exist on a clinical andhistological spectrum.