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Acrokeratoelastoidosis - Skin
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Acrokeratoelastoidosis - Skin

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Contributors: Amanda Robinson MD, Alexa Kimball MD, Jeffrey D. Bernhard MD, Paul Kelly MD, Lowell A. Goldsmith MD, MPH, Lynn McKinley-Grant MD, Aída Lugo-Somolinos MD
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Synopsis

Acrokeratoelastoidosis (AKE) is an uncommon genodermatosis characterized by hyperkeratotic papules coalescing into plaques on the hands and feet. The lesions are asymptomatic and benign. The condition is quite rare, with only a handful of cases reported. It is inherited in an autosomal dominant pattern.

Abnormal and decreased elastic fibers in the dermis cause the characteristic lesions. Among the few scattered cases reported in the literature there was no racial or sex predilection. Age of onset varies widely. Progression of disease has been reported during pregnancy. An association with both localized and systemic scleroderma has been suggested. The current understanding of pathogenesis includes a possible linkage to chromosome 2. An overproduction of filaggrin is thought to accumulate above the granular layer and is incorporated into the protein matrix of epidermal keratin. Nail changes are not usually described with AKE, but there has been at least one report of dystrophic nails in a patient with AKE.

Codes

ICD10CM:
Q82.8 – Other specified congenital malformations of skin

SNOMEDCT:
402773000 – Punctate palmoplantar keratoderma

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Several other conditions should be considered in the differential for smooth yellow papules on the extremities:

Best Tests

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Management Pearls

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Therapy

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References

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Last Updated: 09/04/2013
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Acrokeratoelastoidosis - Skin
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Acrokeratoelastoidosis : Dorsum of foot, Dorsum of hand, Smooth papule
Clinical image of Acrokeratoelastoidosis
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