Amicrobial pustulosis of the folds. Report of 6 cases and a literature review

Abstract

Background: Amicrobial pustulosis of the folds (APF) is a rare entity characterized by relapsing pustular lesions involving mainly the cutaneous folds. The disease typically occurs in the context of an autoimmune disorder and is classified within the spectrum of neutrophilic dermatoses.

Objective: We studied 6 young females having APF associated with various autoimmune diseases or with the presence of serum autoantibodies and reviewed the literature, in order to give a clinical overview on this syndrome.

Methods: Various routine and immunological laboratory tests, histopathological examination as well as direct and indirect immunofluorescence examinations were performed. In vitro neutrophil function was evaluated in 5 cases. We report our findings and compare our cases with those published in the literature.

Results: Clinically, at least one major fold and at least one minor fold as well as the anogenital area were always involved. We documented an impaired neutrophil chemotaxis in 2 subjects, neutrophil dysfunction, thus failing to be a verifying criterion.

Conclusion: APF is a neutrophilic dermatosis affecting young females, which usually shows a benign clinical behavior. Although systemic corticosteroids are the most widely used therapeutic agents, we suggest that the combination of cimetidine and ascorbic acid represents a safe alternative, which may induce long-lasting clinical remission.