A Case of Erythema Elevatum Diutinum associated with Autoimmune Neutropenia and Sjögren Syndrome

Abstract

We report a case of erythema elevatum diutinum (EED) associated with autoimmune neutropenia (AIN) and Sjögren syndrome. The patient was a 71-year-old man who had had Sjögren syndrome for 15 years. He almost simultaneously developed EED and AIN. He was presented with symmetrical erythematous plaques with elevations on the bilateral elbows and knees. The skin biopsy revealed leukocytoclastic vasculitis throughout the dermis. The administration of diaminodiphenyl sulfone was an effective treatment for the skin lesions. To our knowledge, this is the first case of EED associated with AIN and Sjögren syndrome. Although the causes of these three diseases are unknown, this case seems to support the hypothesis of immunological disturbances underlying these diseases.