Amicrobial pustulosis of the folds (APF) is characterized by relapsing, primary aseptic, pustular eruptions mainly affecting the cutaneous folds, scalp, and periorificial regions such as the mouth, external ear canal, and nostrils. APF is a rare condition that has previously been reported in 25 women exhibiting a wide spectrum of autoimmune abnormalities, particularly systemic lupus erythematosus. Histology of the skin lesions usually shows spongiform pustulation in the upper layer of the epidermis and a polymorphonuclear infiltrate in the dermis. APF must be differentiated from other noninfectious pustular diseases such as subcorneal pustulosis (Sneddon-Wilkinson disease) and pustular forms of psoriasis. APF should be included in the spectrum of reactive neutrophilic dermatoses, even though the exact pathomechanisms remain obscure. However, the striking female predominance may be of pathogenetic significance. Based on the small number of previously reported patients, the most effective therapy seems to be medium-dose systemic corticosteroids.