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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. |
