Acrokeratoelastoidosis

Updated: Jun 08, 2016
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD  more...
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Overview

Background

Acrokeratoelastoidosis (AKE) is a rare genodermatosis characterized by small, firm papules or plaques on the sides of the hands and feet. These nodules may result from an abnormality in the secretion or excretion of elastic material by fibroblasts in the dermis. Acrokeratoelastoidosis was first described in 1953 by Costa. [1]

Both autosomal dominant and sporadic forms have been observed. Acrokeratoelastoidosis is not congenital; it slowly arises at puberty, or sometimes later, and then remains stable. Usually, no treatment is necessary. Acrokeratoelastoidosis is similar to 2 other diseases: keratoelastoidosis marginalis [2, 3] and focal acral hyperkeratosis. [4, 5] The clinical and histologic differences among these diseases allow their distinction.

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Pathophysiology

The cause of acrokeratoelastoidosis is not known. Autosomal dominant transmission is common, but the clinical expressions vary widely. Acrokeratoelastoidosis-like lesions on the palms of patients have recently been noted in association with systemic or localized scleroderma, possibly due to an altered pattern of connective tissue metabolism similar to that of systemic scleroderma.

In 2003, Yoshinaga et al reported on a patient with Acrokeratoelastoidosis in association with localized scleroderma. [6]

In 2002, Tajima et al found a high rate of Acrokeratoelastoidosis in patients with systemic scleroderma (7 in 26 systemic sclerodermas). [7]

No other reports have confirmed these findings, and the relationship between these 2 diseases is not conclusive.

A possible linkage to chromosome 2 has also been proposed, [8] but further studies are needed to confirm this hypothesis.

Two cases of unilaterial acrokeratoelastoidosis have been reported. [9]

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Epidemiology

Frequency

United States

Acrokeratoelastoidosis is rare.

International

The eruption is rare, and when the lesions are few, Acrokeratoelastoidosis often remains unnoticed. A survey of 500 consecutive elderly outpatient dermatology patients at the medical school in Puducherry, India found acrokeratoelastoidosis marginalis in 2 (0.9%) females. [10]

Sex

Women appear to be affected more frequently than men.

Age

Acrokeratoelastoidosis is not congenital. It arises at puberty or sometimes later. Some cases have been described in the pediatric dermatologic literature. [11]

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Prognosis

Once present, the eruption is stable, with no adverse effects. Recurrences are common.

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