Acrokeratoelastoidosis of Costa

Definition

Definition of Acrokeratoelastoidosis of Costa
Acrokeratoelastoidosis of Costa (also known as "Keratoelastoidosis marginalis") is a familial condition characterized by multiple keratotic papules on the dorsum of the hands and feet, palms, soles, in which electron microscopy shows rarified, abnormal elastic tissue.

Symptoms

Symptoms of Acrokeratoelastoidosis of Costa
Signs and symptoms of acrokeratoelastoidosis start to arise at around puberty or sometimes later. Most patients slowly develop small bumps over the sides of their hands and feet. The papules are about 2-5mm in diameter and form small clusters. Although the papules look like plane warts they are more keratotic (made of horny skin tissue) and firm, and they do not coalesce. They may sometimes be a yellowish colour.

Once developed the papules of acrokeratoelastoidosis remain unchanged indefinitely.

Causes

Causes of Acrokeratoelastoidosis of Costa
Acrokeratoelastoidosis usually starts in childhood or adolescence and appears to affect females more frequently than males. It is a rare genetic skin disorder with autosomal dominant inheritance patterns (if one parent is affected there is a 50% chance that each child will be affected). However, sporadic cases have also occurred.

Although acrokeratoelastoidosis is genetic in origin the precise cause has not yet been determined. A possible linkage to chromosome 2 has been proposed. In addition it appears to occur in patients with either systemic sclerosis or localised scleroderma, however further studies are required to determine any relationship between the two conditions.

A similar appearance may arise on the sides of the hands in fair skinned patients that have had a lot of exposure to the sun. This is part of the skin ageing process and results from degeneration of the deeper layers of the skin by ultraviolet radiation. It is not inherited. It is known as keratoelastoidosis marginalis, marginal keratoderma of palms and degenerative collagenous plaques of the hands.

Diagnosis

Diagnosis of Acrokeratoelastoidosis of Costa
It is known as keratoelastoidosis marginalis, marginal keratoderma of palms and degenerative collagenous plaques of the hands. The lesions corresponded microscopically to foci of hyperkeratosis and acanthosis. Acid orcein stain revealed marked elastic fiber fragmentation. Acrokeratoelastoidosis of Costa (AKE) was diagnosed. The same damage to the elastic fibers was also present in an additional specimen from grossly uninvolved skin. On electron microscopy there were pronounced changes of the elastic fibers with elastolysis in both specimens.

Prognosis

Prognosis of Acrokeratoelastoidosis of Costa
Consult with your doctor.

Treatment

Treatment of Acrokeratoelastoidosis of Costa
Because acrokeratoelastoidosis is a genetic disorder, no cure is available. Treatment is also not indicated as in most patients, once developed, the eruption is stable and causes no problems. Mild keratolytics such as urea cream have been used, but recurrences are common.

Prevention

Prevention of Acrokeratoelastoidosis of Costa
Consult with your doctor.