AMICROBIAL PUSTULAR DERMATOSIS OF CUTANEOUS FOLDS ASSOCIATED WITH AUTOIMMUNE DISORDERS - A NEW ENTITY

Background: An unclassified amicrobial pustular dermatosis particularl y affecting the main cutaneous folds, external auditory canals and sca lp and coexisting with systemic lupus erythematosus has been recently described. Objective: We studied 3 young females bearing such cutaneou s manifestations in association with subacute cutaneous lupus erythema tosus, celiac disease and various serum autoantibodies, respectively, in order to further characterize this possibly new entity. Methods: Va rious routine and immunological laboratory tests, histopathologic and direct immunofluorescence examinations and in vitro studies of neutrop hil function were performed in each patient. Results: We reported our findings and compared our cases with the few others appearing in the l iterature. We documented an impaired neutrophil chemotaxis in 2 subjec ts, but neutrophil dysfunction does not seem to be one of the verifyin g criteria. Conclusions: All of these cases may represent a distinctiv e form in the clinicopathological spectrum of neutrophilic dermatoses (ND) because of the typical distribution and the close link with diffe rent autoimmune disorders. Cimetidine in combination with ascorbic aci d can be indicated as an effective and safe alternative to the classic medications of ND, although the action of both drugs remains unexplai ned.