Pemphigus herpetiformis is a rare clinical expression of nonendemic pemphigus foliaceus, fogo selvagem, and pemphigus vulgaris. Cooperative Group on Fogo Selvagem Research.
BACKGROUND
Pemphigus herpetiformis is a rare and atypical variant of pemphigus that resembles dermatitis herpetiformis. Most patients show antiepidermal autoantibodies that stain the epidermal intercellular spaces by immunofluorescence, similar to pemphigus autoantibodies, and lack the immunopathologic features of dermatitis herpetiformis.


OBJECTIVE
The study was aimed at characterizing the specificity of the antiepidermal autoantibodies in seven patients with pemphigus herpetiformis.


METHODS
The antiepidermal autoantibodies were characterized by immunofluorescence, immunoblotting, and immunoprecipitation studies in seven patients who fulfilled the clinical, histologic, and immunofluorescence diagnostic criteria for pemphigus herpetiformis.


RESULTS
Five patients with features of pemphigus herpetiformis either had classic pemphigus foliaceus, or their disease evolved into classic pemphigus foliaceus. One of these patients had fogo selvagem. Two of the seven patients showed features of or had disease that evolved into pemphigus vulgaris. The antiepidermal autoantibodies present in all seven patients recognized desmoglein 1.


CONCLUSIONS
Pemphigus herpetiformis is a rare clinical and histologic expression of nonendemic pemphigus foliaceus, fogo selvagem, and pemphigus vulgaris.
Department of Dermatology, University of São Paulo.
C G
CG
Santi
C W
CW
Maruta
V
V
Aoki
M N
MN
Sotto
E A
EA
Rivitti
L A
LA
Diaz
D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000032:analysis*; D003598:Cytoskeletal Proteins / Q000276:immunology; D051183:Desmoglein 1; D051182:Desmogleins; D051180:Desmoplakins; D003896:Desmosomes / Q000276:immunology; D004817:Epidermis / Q000276:immunology*; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D015151:Immunoblotting; D008297:Male; D008875:Middle Aged; D010392:Pemphigus / Q000276:immunology* / Q000473:pathology; D011233:Precipitin Tests
D001323:Autoantibodies; D003598:Cytoskeletal Proteins; C495297:DSG1 protein, human; D051183:Desmoglein 1; D051182:Desmogleins; D051180:Desmoplakins