Angiokeratomas, acral pseudolymphomatous childhood D23.9

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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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.

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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.

Clinical features
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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 inflammatory (non-epidermotropic) infiltrate mainly from CD3+ T lymphocytes, a few B lymphocytes, macrophages and plasma cells in the upper and middle dermis. Occasionally also eosinophil granulocytes and multinuclear giant cells. Ample vascular incisions with partly prominent endothelia. Formation of lymph follicles possible.

Differential diagnosis
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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. Gansz B et al (2005) Acral pseudolymphomatous angiokeratoma of children. dermatologist 56: 270-272
  3. 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
  4. Lee MW et al (2003) Acral pseudolymphomatous angiokeratoma of children (APACHE). Pediatric dermatol 20: 457-458
  5. Murakami T et al (2001) Acral pseudolymphomatous anguikeratoma of children: a pseudolymphoma rather than an angiokeratoma. Br J DErmatol 145: 512-514
  6. Ramsay B, Dahl MC, Malcolm AJ, Wilson-Jones E (1990) Acral pseudolymphomatous angiokeratoma of children. Arch Dermatol 126: 1524-1555
  7. Wagner G (2014) Papular pseudolymphomas in adulthood as a variant of the acral pseudolymphomatous angiolkeratomas of childhood (APACHE)

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Last updated on: 29.10.2020