Acrokeratoelastoidosis

SUMMARY A case of acrokcratoelastoidosis (AKE) is reported. A review of the literature has established that at least three clinicopathotogical entities have been reported under this term. Two of the entities show a combination of hyperkcratosis with fragmentation of dermal elastic tissue, which justifies the designation AKE. The first, for which the term familial AKE is perhaps appropriate, though many cases have been sporadic, begins in childhood or early adult life, and there is no evidence that trauma or light exposure are factors in its production. The second is a more variable clinical syndrome occurring in middle or late adult life, and both physical trauma and light exposure appear to be concerned in its induction. The third syndrome to be reported under the designation AKE shows no abnormality of elastic tissue and appears to be a separate entity.