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Review
. 2013 Nov-Dec;88(6 Suppl 1):39-43.
doi: 10.1590/abd1806-4841.20132413.

Acral pseudolymphomatous angiokeratoma: case report and literature review

Priscila Pacheco Lessa  1 , Juliana Chaib Ferreira Jorge  1 , Flávia Regina Ferreira  2 , Marcia Lanzoni de Alvarenga Lira  3 , Samuel Henrique Mandelbaum  4
Affiliations
Review

Acral pseudolymphomatous angiokeratoma: case report and literature review

Priscila Pacheco Lessa et al. An Bras Dermatol. 2013 Nov-Dec.

Abstract
in English, Portuguese

The authors describe a case of a female patient with Acral Pseudolymphomatous Angiokeratoma of Children, known as APACHE. It is a rare benign cutaneous disease, of unknown etiology, characterized by multiple, asymptomatic erythematous-violaceous papules and nodules, usually located unilaterally with acral distribution. Today, this denomination is questionable, since there are published reports of this disease in adults and in different locations. Clinically, it is similar to an angiokeratoma, whereas hystologically, it corresponds to a distinct type of pseudolymphoma. The immunohistochemical study is required to distinguish APACHE from cutaneous lymphoma.

Relata-se o caso de uma paciente com diagnóstico de angioqueratoma pseudolinfomatoso acral, conhecido por sua sigla em inglês APACHE - Acral Pseudolymphomatous Angiokeratoma of Children. É uma doença cutânea benigna, rara, de etiologia desconhecida, caracterizada por múltiplas pápulas e nódulos eritêmato-violáceos assintomáticos, de localização geralmente unilateral e acral. Atualmente, questiona-se esta denominação, já que há relatos na literatura do quadro em adultos e em outras localizações. Clinicamente, é similar a um angioqueratoma, porém, histologicamente, corresponde a um tipo distinto de pseudolinfoma. O estudo imuno-histoquímico é necessário para diferenciação dos linfomas cutâneos.

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Conflict of interest statement

Conflict of Interest: None

Figures

FIGURE 1
FIGURE 1
Left wrist - grouped erythematous-violaceous papules
FIGURE 2
FIGURE 2
Dense lymphomononuclear infiltrate of plexiform pattern (HE, 40x)
FIGURE 3
FIGURE 3
A. Infiltrate occupying the deep dermis (HE, 100x); B. Infiltrate composed of plasmocytes (HE, 400x)
FIGURE 4
FIGURE 4
Immunohistochemical panel: A. Anti-CD20 (400x). B. Anti-CD8 (400x)
See this image and copyright information in PMC

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