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Angiokeratomas, acral pseudolymphomatous childhoodD23.9
Synonym(s)
acral angiokeratomatous pseudolymphoma; acral pseudolymphomatous angiokeratoma of childhood; acral pseudolymphomatous angiokeratoma of children; APACHE; Childhood acral pseudo-lymphomatous angiokeratomas; small papular pseudolymphoma
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.
ClinicThis 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 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.
Differential diagnosisThis section has been translated automatically.
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.
- Crow KD (1980) Case for diagnosis. Br J Dermatol 103: 78-80
- 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
- Gansz B et al. (2005) Acral pseudolymphomatous angiokeratoma of children. Dermatology 56: 270-272
- 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
- Lee MW et al. (2003) Acral pseudolymphomatous angiokeratoma of children (APACHE). Pediatr Dermatol 20: 457-458
- Lessa PP et al. (2013) Acral pseudolymphomatous angiokeratoma: case report and literature review. An Bras Dermatol 88(6 Suppl 1):39-43.
- Murakami T et al. (2001) Acral pseudolymphomatous angiokeratoma of children: a pseudolymphoma rather than an angiokeratoma. Br J DErmatol 145: 512-514
- Ramsay B, Dahl MC, Malcolm AJ, Wilson-Jones E (1990) Acral pseudolymphomatous angiokeratoma of children. Arch Dermatol 126: 1524-1555
- Wagner G (2014) Papular pseudolymphoma in adulthood as a variant of acral pseudolymphomatous angiokeratoma of childhood (APACHE)