Marginal acrokeratoderma: a case series

Abstract

<p>Marginal acrokeratoderma (MAK) are complex disorders characterized by distribution of cornified papules/plaques along the dorso-plantar and dorso-palmar junction of the feet and hands. They belong to the family of punctate palmoplantar keratoderma. No associated gene defect has been detected. There are two clinically identical types of hereditary/familial MAK; namely acrokeratoelastoidosis (AKE) and focal acral hyperkeratosis (FAH); differentiated by the presence of elastorrehexis in AKE. The aim of this article is to report MPK lesions that are not included in the familiar groupings of punctate and polygonal papules. We described thirteen cases and reviewed the literature. Average age at presentation was 24 years, age ranged from 5 to 79 years. Majority of our cases had lesions on only their feet, that of FAH had lesions on only their hands and AKE had lesions on both hands and feet. Although most lesions reported in literature with FAH and AKE were characterized as 2-5mm flesh-colored to yellowish flat-topped/polygonal, keratotic/punctate papules, coalescing into plaques, we also reported lesions that were keratotic with cracks, desquamating and cobble-stone in appearance. This case series calls for more studies on variations in clinical presentation of MAK lesions and an opportunity to revisit the genetic basis and investigate triggers of this rare disorder.</p>

Disorders of Keratinization

“…Laser therapy, topical corticosteroids, topical and systemic retinoids, cryotherapy, and topical keratolytics are used for its treatment (Bhobe et al 2016). -Differential diagnosis: Different disorders with manifestations of marginal papular keratoderma including hereditary papulotranslucent acrokeratoderma, mosaic acral keratosis, digital popular calcinosis, degenerative collagenous plaques of the hands, and keratoelastoidosis marginalis (Rongioletti et al 1994), and acrokeratosis verruciformis of Hopf, colloid milium, and plane warts (Ibekwe and Ogunbiyi 2020). Acquired keratodermas show different patterns of epidermal involvement including diffuse, focal, and punctate.…”