Acrokeratoelastoidosis Workup

Updated: Apr 07, 2021
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD  more...
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Laboratory Studies

Laboratory studies or other studies are usually not necessary. Biopsy can be helpful for diagnostic purposes and for ruling out other disorders.


Histologic Findings

The typical epidermal features of acrokeratoelastoidosis (AKE) are hyperkeratosis with hypergranulosis, elastorrhexis, acanthosis, and epidermal hyperplasia. [22] Some of these findings may be absent.

The changes in the dermal elastic fibers are characteristic. The number of elastic fibers is often diminished, and the fibers have a discrete-to-intense fragmentation. An elastic fiber stain, such as the Weigert, Verhoeff, or orcein stain, may be useful in demonstrating this fragmentation. Periodic acid-Schiff (PAS) is used to stain carbohydrates, and occasionally, the PAS test is used to rule out other conditions. The cell walls of fungi are rich in carbohydrates, and they stain positively with PAS. PAS does not stain elastic fibers. Acrokeratoelastoidosis has been evaluated ultrastructurally. [23]