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


CONCLUSIONS
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.
Marzano
Angelo V
AV
Institute of Dermatological Sciences, University of Milan - Fondazione IRCCS, Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena, Milan, Italy. francesca.alessi@unimi.it
Ramoni
Stefano
S
Caputo
Ruggero
R
D000328:Adult; D000367:Age Factors; D001327:Autoimmune Diseases / Q000150:complications* / Q000473:pathology; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D008178:Lupus Erythematosus, Cutaneous / Q000276:immunology; D009504:Neutrophils / Q000276:immunology / Q000473:pathology; D012737:Sex Factors; D012871:Skin Diseases / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D013577:Syndrome