Marginal Papular Acrokeratodermas: A Unified Nosography for Focal Acral Hyperkeratosis, Acrokeratoelastoidosis and Related Disorders

Abstract

Several entities, quite often similar, share keratotic papules, usually crateriform, along the border of the hands and feet as a common clinical finding. These conditions are acrokeratoelastoidosis of Costa, focal acral hyperkeratosis, acrokeratoelastoidosis of Matthews and Harman, mosaic acral keratosis, hereditary papulotranslucent acrokeratoderma, acrokeratoderma hereditarium punctatum, degenerative collagenous plaques of the hands, keratoelastoidosis marginalis and digital papular calcinosis. We describe two further cases of focal acral hyperkeratosis in two Caucasian women, review the clinical and histological features of the related conditions and suggest that some are variants of the same entity. We propose the simplified concept of marginal papular acrokeratodermas that may be divided into (1) the hereditary type (subdivided into that with and without elastorrhexis) and (2) the acquired type.

A Case of Focal Acral Hyperkeratosis

“…Both sporadic and familial forms have been reported, with an apparent autosomal dominant inheritance in the familial cases. FAH is clinically similar to acrokeratoelastoidosis (AKE); both belong to the group of marginal papular acrokeratodermas (MPA) 2 . The histopathology, however, reveals subtle differences between the two; FAH does not have elastorrhexis.…”

Marginal Papular Acrokeratodermas: No Racial Limitations for a Clinical Spectrum That Responds to Acitretin

“…For years, it has been considered a racially limited [1]focal disorder of keratinization and pigmentation [2], found in people of Afro-Caribbean or Arabian origin, although 2 Caucasian cases were reported by Rongioletti et al [3]in 1994, who first proposed the simplified concept of MPA.…”

Acrokeratoelastoidosis successfully treated with 10% salicylic acid ointment

“…Therefore, Rongioletti et al 3 have proposed the term "marginal popular acrokeratoderma (MPA)" to encompass these. We herein report a case of acrokeratoelastoidosis successfully treated with 10% salicylic acid ointment.…”