Angiokeratomas, acral pseudolymphomatous childhoodD23.9

Author:Prof. Dr. med. Peter Altmeyer

All authors of this article

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

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HistoryThis section has been translated automatically.

Crow, 1980; Ramsay, 1988

DefinitionThis section has been translated automatically.

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

Occurrence/EpidemiologyThis section has been translated automatically.

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

EtiopathogenesisThis section has been translated automatically.

Unknown; post-traumatic?

ManifestationThis section has been translated automatically.

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

LocalizationThis section has been translated automatically.

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

Clinical featuresThis section has been translated automatically.

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

HistologyThis section has been translated automatically.

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 diagnosisThis section has been translated automatically.

Angiokeratoma Mibelli; acroangiodermatitis; hemangiomas; nevus, epidermal; lichen aureus; granuloma pyogenicum; eczema, contact dermatitis

TherapyThis section has been translated automatically.

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

Progression/forecastThis section has been translated automatically.

Benign; years of persistence; spontaneous remissions are described.

LiteratureThis section has been translated automatically.

  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)

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer