Acrokeratoelastoidosis

  

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

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 and focal acral

hyperkeratosis.The clinical and histologic differences

 among these diseases allow their distinction

 

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. In 2002, Tajima et al found a high rate of Acrokeratoelastoidosis in patients with systemic scleroderma (7 in 26 systemic sclerodermas). 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, but further studies are needed to confirm this hypothesis

 

 

Frequency

United States

Acrokeratoelastoidosis is rare.

International

The eruption is rare, and when the lesions are few, Acrokeratoelastoidosis often remains unnoticed.

Mortality/Morbidity

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

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.⁹

History

• 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.
• 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 2 diseases is not conclusive.

Physical

• 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 1 or both feet are the only areas affected. In rare instances, the lesions spread to the dorsum of the hands, feet, or both.
• 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.
• A case of acrokeratoelastoidosis has been seen in association with nail dystrophic changes. Further observations may lead to the definition of a new entity.¹⁰

Causes

• No local or systemic causes have been identified.
• Autosomal dominant transmission is common.
• Sporadic cases of acrokeratoelastoidosis are also described.

One report described a patient with endogenous ochronosis showing clinical features similar to acrokeratoelastoidosis.

Treatment

Medical Care

• Treatment is not indicated in most patients.

• Mild keratolytics occasionally help, but recurrences are common.

• Topical retinoids are not effective.

• The erbium:YAG laser has been effective in improving one patient. No recurrence developed during afollow-up of 6 months