Acral pseudolymphomatous angiokeratoma of children (APACHE)

Abstract

13-year-old girl presented with a 10-year history of multiple discrete red firm papules situated on her right upper arm. The histopathology examination revealed a massive subepidermal lymphohistiocytic infiltrate, consistent with acral pseudolymphomatous angiokeratoma of children. Treatment with topical application of mometasone furoate 0.1% cream once daily for 6 months improved the lesions partially. However, these papules returned to their pretreatment appearance and consistency within 6 months after the cessation of the topical corticosteroid. The authors prefer the term 'papular angiolymphoid hyperplasia' for this entity, based on the absence of histological angiokeratomatous features, the benign nature of the lesion and the previously reported cases from adults and non-acral sites.

Acral pseudolymphomatous angiokeratoma of children in an elderly man: report of a case and review of the literature

“…Single lesions as well as lesions assuming a linear configuration 6,7 or affecting the trunk have been reported 3 . Interestingly, most case reports originated from Japan and revealed a female predominance 8 …”

Acral Pseudolymphomatous Angiokeratoma of Children

“…The features of all cases reported to date are presented in Table 1. [6][7][8][9][10][11][12][13][14][15][16][17][18] Seventy-seven percent of patients were female. Acral lesions (foot and hand) were seen in 52% of cases, and 97% of cases included limb lesions.…”

Papular pseudolymphoma of adults as a variant of acral pseudolymphomatous angiokeratoma of children (APACHE)

“…Initially, Ramsay et al thought that vascular tumors (angiokeratomas) and an associated nevoid disorder might be the origin of the disease [1]. Currently, the majority of the authors supports an inflammatory origin and believes APACHE to be a variant of pseudolymphoma [3][4][5]. Its exact cause is still unknown.…”

“…Another advantage of this name is that it does not refer to a special localization or age group. Small papular pseudolymphoma [8] Small lymphoid papules of the extremities [8] Papular angiolymphoid proliferation with epithelioid features in adults and children [9] Angiolymphoid hyperplasia with high endothelial venules [10] Acral angiokeratoma-like pseudolymphoma [11] Papular angiolymphoid hyperplasia [4] Angiolymphoid hyperplasia [5] Pseudolymphomatous angiokeratoma [3] …”