Early lesions show vasculitis in the small vessels of the upper and mid-dermis. The infiltrate may accumulate between collagen bundles. Granulation tissue and fibrosis in the dermis characterize older lesions. Furthermore, there is a tendency towards limiting the use of the term extracellular cholesterosis, since it is now believed that the main lipid deposits are intracellular and that they are formed of cholesterol esters produced by damaged tissue. Several studies and clinical experience have shown good response of the disease to dapsone, therefore making it the treatment of choice.
Dosage depends on each patient.
Sulfones have a suppressive effect on EED as shown by the recurrence of the disease after drug withdrawal. Systemic steroids generally have not been found to be effective. Sulfapyridine has had similar effects as dapsone. The IgA levels responded to PLEX treatment followed by consolidative doses of cyclophosphamide. This treatment might be promising for the control of severe EED that is not controlled by dapsone.