Acrokeratoelastoidosis Clinical Presentation

Updated: Apr 07, 2021
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD  more...
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The patient may complain of the gradual onset of small bumps over the margins of the hands and feet. After onset, the eruption remains stable indefinitely. Acrokeratoelastoidosis (AKE) evident as a unilateral sporadic keratosis on the hand and foot has been described in a prepubescent child. [13]

No local or systemic symptoms are associated with acrokeratoelastoidosis. Some reports show acrokeratoelastoidosis in patients with localized or systemic scleroderma, but the relationship between these two diseases is not conclusive. There has been no established association between AKE and asthma and sporadic cases in children, although one has been suggested. [14]


Physical Examination

A cluster of small, discrete, grouped papules characterizes acrokeratoelastoidosis. The papules are usually 2-5 mm in diameter and often occur in a linear distribution. These small round-oval– to rhomboid–shaped yellowish papules are most commonly localized to the palmar surfaces of the hands and, sometimes, on the plantar surfaces of the feet. The margins of both hands and one or both feet are the only areas affected. In rare instances, the lesions spread to the dorsum of the hands, feet, or both. See the image below. Dermatoscopic examination may show yellowish, structureless, linear areas. [15]

Acrokeratoelastoidosis. Courtesy of William D Jame Acrokeratoelastoidosis. Courtesy of William D James, MD.

The papules resemble plane warts, but they are more keratotic and firm; they do not coalesce. Some translucency is often evident. Occasionally, just a few papules are present. It may rarely appear as plaques. [16]

A case of acrokeratoelastoidosis (AKE) has been seen in association with nail dystrophic changes. Further observations may lead to the definition of a new entity. [17]



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