Primary anetoderma and antiphospholipid antibodies--review of the literature.

Primary anetoderma (PA) is a rare elastolytic disorder that develops in clinically normal skin or following a nonspecific inflammatory process. The old literature contains numerous reports of the association between PA and lupus eryhtematosus, although the relationship has not been clearly established. In recent years, there has been a growing body of literature linking PA with a wide range of immunologic abnormalities, the most common of which is the presence of antiphospholipid antibodies, with or without antiphospholipid syndrome. The present review summarizes the literature, from the early descriptions pointing toward an immunologic basis of PA and up to the present recognition that PA is a cutaneous sign for autoimmune disorders, in general, and the presence of antiphospholipid in particular.