“African” acral keratoderma: Further evidence for a single entity. A retrospective study of 42 patients

Abstract

BackgroundSeveral phenotypes of non-inflammatory palmar and plantar keratoderma (PPK) have been described in patients of Sub-Saharan African descent. They include keratosis punctata of the palmar creases, marginal keratoderma, also known as acrokeratoelastoidosis or focal acral hyperkeratosis, knuckle pads, other forms of diffuse hyperkeratosis, the very rare “mosaic acral keratosis”, and ainhum. A previous survey has shown that these various forms of PPK are particularly frequent in patients of Sub-Saharan African descent and that they commonly occur concurrently, suggesting that they could form part of a single entity called “African” Acral Keratoderma (AAK). AimTo assess the validity of the concept of AAK and clarify its main characteristics. MethodsA retrospective, descriptive, monocenter study was carried out on patients with AAK seen at our institution between 2009 and 2020. ResultsThere were 42 patients (median age 38 years, range: 12–69 years), all of Sub-Saharan African descent. The male–female sex ratio was 0.3. Thirty-three (78%) had diffuse keratoderma, 25 (59%) had marginal keratoderma on their hands and/or feet, 20 (48%) had knuckle pads, 20 (48%) had keratosis punctata of the palmar creases, 3 had ainhum, and 2 had mosaic acral keratoderma. Mixed forms were seen in 76% of the patients (n = 32). Familial histories were reported by 17 patients (40%). Treatment was topical in over 90% of patients and systemic in 9 patients (21%). Ainhum was managed surgically. ConclusionThis retrospective study provides additional evidence for the concept of AAK. A genetic origin is suggested by the familial aggregation of cases.