Both autosomal dominant and sporadic forms have been observed. AKE is not congenital; it slowly arises at puberty, or sometimes later, and then remains stable. Usually, no treatment is necessary. AKE 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 AKE is not known. Autosomal dominant transmission is common, but the clinical expressions vary widely. AKE-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 AKE in association with localized scleroderma. In 2002, Tajima et al found a high rate of AKE 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 (Greiner, 1983), but further studies are needed to confirm this hypothesis.
Frequency:
- In the US: AKE is rare.
- Internationally: The eruption is rare, and when the lesions are few, AKE 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.
Treatment
Medical Care:
- Treatment is not indicated in most patients.
- Mild keratolytics occasionally help, but recurrences are common.
- Topical retinoids are not effective.
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