Amicrobial pustular dermatosis of cutaneous folds associated with autoimmune disorders: a new entity?
BACKGROUND
An unclassified amicrobial pustular dermatosis particularly affecting the main cutaneous folds, external auditory canals and scalp and coexisting with systemic lupus erythematosus has been recently described.


OBJECTIVE
We studied 3 young females bearing such cutaneous manifestations in association with subacute cutaneous lupus erythematosus, celiac disease and various serum autoantibodies, respectively, in order to further characterize this possibly new entity.


METHODS
Various routine and immunological laboratory tests, histopathologic and direct immunofluorescence examinations and in vitro studies of neutrophil function were performed in each patient.


RESULTS
We reported our findings and compared our cases with the few others appearing in the literature. We documented an impaired neutrophil chemotaxis in 2 subjects, but neutrophil dysfunction does not seem to be one of the verifying criteria.


CONCLUSIONS
All of these cases may represent a distinctive form in the clinicopathological spectrum of neutrophilic dermatoses (ND) because of the typical distribution and the close link with different autoimmune disorders. Cimetidine in combination with ascorbic acid can be indicated as an effective and safe alternative to the classic medications of ND, although the action of both drugs remains unexplained.
Marzano
A V
AV
Institute of Dermatological Sciences, University of Milan, IRCCS, Italy.
Capsoni
F
F
Berti
E
E
Gasparini
G
G
Bottelli
S
S
Caputo
R
R
D000328:Adult; D001205:Ascorbic Acid / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood; D001327:Autoimmune Diseases / Q000150:complications* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D002446:Celiac Disease / Q000150:complications; D002634:Chemotaxis, Leukocyte; D002927:Cimetidine / Q000627:therapeutic use; D004424:Ear Canal / Q000473:pathology; D005260:Female; D019085:Fluorescent Antibody Technique, Direct; D006635:Histamine H2 Antagonists / Q000627:therapeutic use; D006801:Humans; D008178:Lupus Erythematosus, Cutaneous / Q000150:complications* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D009504:Neutrophils / Q000276:immunology / Q000502:physiology; D012536:Scalp Dermatoses / Q000150:complications / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D012867:Skin / Q000473:pathology; D012872:Skin Diseases, Vesiculobullous / Q000150:complications* / Q000188:drug therapy / Q000276:immunology / Q000473:pathology
D001323:Autoantibodies; D006635:Histamine H2 Antagonists; D002927:Cimetidine; D001205:Ascorbic Acid