A Case Series of Amicrobial Pustulosis of Folds: An Eye-opener for the Diagnosis of Autoimmune Disorders

Abstract

Amicrobial Pustulosis of Folds (APF) is a rare, chronic, relapsing cutaneous disease seen exclusively in younger women with a history of autoimmune disease, most commonly Systemic Lupus Erythematosus (SLE), or who simply have circulating autoantibodies. This case series highlights the occurrence of an unusual manifestation of APF as a marker of autoimmune disorders and emphasises the importance of double-stranded Deoxyribose Nucleic Acid (dsDNA) positivity in such cases. A total of five female patients with APF were included in this case series. All five cases presented with pustular lesions lasting 10-12 weeks, involving conchal bowls, eyes, perineal, and perianal regions, either as an initial presentation or as a flare-up of pre-existing autoimmune disease. All patients underwent screening for autoimmune disorders, which revealed dsDNA positivity and met the diagnostic criteria for APF. Therefore, a diagnosis of APF with SLE was made. All patients showed significant improvement with oral steroids. Although SLE is a complex multisystem disorder where patients may not always present with malar rash, photosensitivity, arthritis, and arthralgia, APF could serve as an eye-opener for diagnosing underlying autoimmune diseases.

Keywords

• neutrophilic dermatosis
• pustular dermatosis
• sterile pustules
• systemic lupus erythematosus