Angiokeratomas, acral pseudolymphomatous childhood D23.9

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 26.05.2025

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Synonym(s)

acral angiokeratomatous pseudolymphoma; acral pseudolymphomatous angiokeratoma of childhood; acral pseudolymphomatous angiokeratoma of children; APACHE; Childhood acral pseudo-lymphomatous angiokeratomas; small papular pseudolymphoma

History
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Crow, 1980; Ramsay, 1988

Definition
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Rare, predominantly acral localized, highly vascularized pseudolymphoma occurring under the clinical aspect of a vascular tumor.

Occurrence/Epidemiology
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Very rare. So far, 18 case descriptions under different synonyms have been described in the literature.

Etiopathogenesis
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Unknown; post-traumatic?

Manifestation
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Predilection age: 3-17 years of age. Rarely also in adults.

Localization
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Mainly acral on hands and feet, more rarely on the back, chest or lower legs.

Clinic
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Mostly multiple, partly isolated standing, partly aggregated, 0.1-0.3 cm large, red-livid, smooth or slightly scaling papules.

Histology
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Nodular lymphoid cell (non-epidermotropic) infiltrate in the upper and middle dermis, predominantly consisting of CD3+ T lymphocytes, a few B lymphocytes as well as macrophages and plasma cells. Occasionally also eosinophilic granulocytes and multinuclear giant cells. Abundant vascular incisions with partly prominent endothelia. Formation of lymph follicles possible.

Therapy
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For isolated tumors excision or curettage. External or intrafocal application of glucocorticoids leads only to short-term remissions but also to regular relapses.

Progression/forecast
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Benign; years of persistence; spontaneous remissions are described.

Literature
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  1. Crow KD (1980) Case for diagnosis. Br J Dermatol 103: 78-80
  2. Fernandez-Flores A et al. (2015) Expression of WT-1 by the vascular component of acral pseudolymphomatous angiokeratoma of children. J Cutan Pathol 42(1):50-5
  3. Gansz B et al. (2005) Acral pseudolymphomatous angiokeratoma of children. Dermatology 56: 270-272
  4. Kiyohara T et al. (2003) Linear acral pseudolymphomatous angiokeratoma of children (APACHE): further evidence that APACHE is a cutaneous pseudolymphoma. J Am Acad Dermatol 48(2 Suppl): S15-17
  5. Lee MW et al. (2003) Acral pseudolymphomatous angiokeratoma of children (APACHE). Pediatr Dermatol 20: 457-458
  6. Lessa PP et al. (2013) Acral pseudolymphomatous angiokeratoma: case report and literature review. An Bras Dermatol 88(6 Suppl 1):39-43.
  7. Murakami T et al. (2001) Acral pseudolymphomatous angiokeratoma of children: a pseudolymphoma rather than an angiokeratoma. Br J DErmatol 145: 512-514
  8. Ramsay B, Dahl MC, Malcolm AJ, Wilson-Jones E (1990) Acral pseudolymphomatous angiokeratoma of children. Arch Dermatol 126: 1524-1555
  9. Wagner G (2014) Papular pseudolymphoma in adulthood as a variant of acral pseudolymphomatous angiokeratoma of childhood (APACHE)

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Last updated on: 26.05.2025