ANGIOKERATOMA CIRCUMSCRIPTUM NAEVIFORME

By Gary M. White, MD


The Angiokeratoma Circumscriptum Naeviforme (ACN) is a term used to describe a larger vascular plaque, usually congenital and most commonly occurring on the legs. It typically becomes verrucous over time. The term verrucous hemangioma (VH) has been used to describe a similar entity. Supposedly though, they can be distinguished histologically.

The VH is a congenital, localized, vascular malformation, histopathologically manifesting as dilated capillaries and large endothelial-lined, blood-filled spaces extending well into the reticular dermis and subcutaneous tissue with an overlying hyperkeratosis. Histologically, the ACN does not demonstrate the deep angiomatous component characteristic of the VH. Instead, it shows numerous dilated capillaries in the papillary dermis.

Clinical

Both entities appear very similar clinically. A congenital vascular plaque or plaques of the lower legs which become(s) verrucous over time is typical.

Treatment

For small lesions, local destruction with electrocautery and/or laser may be tried. For larger lesions, surgical excision is the mainstay of therapy, but can be challenging depending upon size and there is a high recurrence rate. Surgery and laser therapy may be combined. The long pulse alexandrite laser has been used successfully [JEADV 2017;31;e186].

References

Indian Dermatol Online J. 2014 Oct-Dec; 5(4): 472–474

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