Amicrobial Pustulosis of the Folds Associated with Autoimmune Disorders: Systemic Lupus Erythematosus Case Series and First Report on the Association with Autoimmune Hepatitis

Abstract

Amicrobial pustulosis of the folds (APF) associated with autoimmune disorders is an infrequent entity characterized by the recurrent appearance of follicular and nonfollicular sterile pustules in the context of autoimmune disease. Most reports on APF suggest systemic lupus erythematosus (SLE) as the major immunological associated disorder but the association with autoimmune hepatitis (AH) has not been previously documented. We describe the clinical and histological characteristics of 5 patients with APF: 4 with SLE and 1 with AH. As APF is an exclusion diagnosis, in order to establish an opportune diagnosis and treatment, physicians should be aware of patients with any autoimmune disease who develop a pustular dermatosis for which cultures and stains are negative. We propose the inclusion of anti-liver kidney microsome antibodies in the minor criteria for APF diagnosis.