Acrokeratoelastoidosis Differential Diagnoses

Updated: Apr 07, 2021
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD  more...
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Diagnostic Considerations

Keratoelastoidosis marginalis, also called degenerative collagenous plaques of the hands, is related to sun exposure or local trauma. [2, 3]

Bothnia-type palmoplantar keratoderma has been described with acrokeratoelastoidosis (AKE)–like features linked with AQP5 mutations. [18]

Focal acral hyperkeratosis has no dermal changes, though these are characteristic features of acrokeratoelastoidosis. [4, 5]

Aquagenic palmoplantar keratoderma has been described associated with acrokeratoelastoidosis. [19]

There are a variety of African acral keratoderma, some with ainhum, that should also be considered. [20]

Olmsted syndrome, a rare entity with bilateral mutilating transgredient palmoplantar keratoderma and periorificial keratotic plaques, may also require distinction. [21] It may be associated with pseudoainhum and hair and nail abnormalities, leukokeratosis, corneal default, and recurrent infections.

Differential Diagnoses