Acrokeratoelastoidosis in Child
Synopsis

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
Q82.8 – Other specified congenital malformations of skin
SNOMEDCT:
111029001 – Acrokerato-elastoidosis
Look For
Subscription Required
Diagnostic Pearls
Subscription Required
Differential Diagnosis & Pitfalls
Subscription Required
Best Tests
Subscription Required
Management Pearls
Subscription Required
Therapy
Subscription Required
References
Subscription Required