Av. Marzano et al., AMICROBIAL PUSTULAR DERMATOSIS OF CUTANEOUS FOLDS ASSOCIATED WITH AUTOIMMUNE DISORDERS - A NEW ENTITY, Dermatology, 193(2), 1996, pp. 88-93
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.