Acrokeratoelastoidosis in Adult
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.
Q82.8 – Other specified congenital malformations of skin
111029001 – Acrokerato-elastoidosis
Differential Diagnosis & Pitfalls