Aseptic Pustulosis of the Folds

Abstract

Aseptic (or amicrobial) pustulosis of the skin folds (APF) is a rare neutrophilic dermatosis, occurring mostly in young females affected with autoimmune or dysimmune disorders, characterized by relapsing sterile pustular eruptions mainly involving the skin folds. The disease was first reported in 1991 by Crickx and colleagues [1], who described two young women with systemic lupus erythematosus (SLE) and outbreaks of amicrobial pustules involving the scalp, major folds, and external ear canals. Subsequently, two other similar cases have been described in association with SLE and incomplete SLE, respectively [2, 3]. On reporting three additional cases in young women with subacute cutaneous lupus erythematosus (SCLE), celiac disease and various serum autoantibodies, respectively, Marzano et al. speculated that APF may represent a new entity within the spectrum of neutrophilic dermatoses [4]. Moreover, the same authors emphasized the concept that this form may be associated not only with lupus, but also with a broad spectrum of underlying autoimmune diseases or immunological abnormalities [4]. Since then, similar clinical features have been described in association with a number of other autoimmune disorders [5–13]. More recently, the inclusion of APF within the spectrum of autoinflammatory diseases has opened a new prospective in the pathophysiology of this condition [14], paving the way to the use of biologic agents targeting the main pro-inflammatory cytokines for the management of refractory or highly relapsing APF cases.