Acrokeratoelastoidosis of Costa: a primary disease of the elastic tissue?

A 24-year-old black woman from Uganda was seen for treatment of multiple pa pules on her hands and feet. The lesions corresponded microscopically to fo ci of hyperkeratosis and acanthosis. Acid orcein stain revealed marked elas tic fiber fragmentation. Acrokeratoelastoidosis of Costa (AKE) was diagnose d. The same damage to the elastic fibers was also present in an additional specimen from grossly uninvolved skin. On electron microscopy there were pr onounced changes of the elastic fibers with elastolysis in both specimens. This case with generalized damage of the dermal elastic tissue supports the view that elastorrhexis is the key feature of AKE. Accordingly, the diseas e could be regarded as a primary elastic tissue disorder. The marginal acra l keratoderma that is seen in AKE patients could represent epidermal change s secondary to chronic trauma.