Mucocutaneous pemphigus vulgaris carrying high-titre antidesmoglein 1 antibodies with skin lesions resembling pemphigus erythematosus.
Patients with pemphigus vulgaris (PV) who have both antidesmoglein (Dsg)1 and anti-Dsg3 antibodies usually develop flaccid blisters on skin and mucous membranes. We report a case of PV with crusting skin lesions resembling pemphigus erythematosus, the localized variant of pemphigus foliaceus (PF). Notably, the patient had high titres of anti-Dsg1 IgG, as assessed by ELISA. We then established an in vitro model of pemphigus, and found that patient's serum was able to induce suprabasilar acantholysis in mouse skin culture. However, epidermal splitting also occurred within the granular layer, suggesting that the pathogenic potential of such a high-titre anti-Dsg1 serum was intermediate between PV and PF. Thus, the levels of anti-Dsg1 antibodies could play a role in determining the clinical phenotype of pemphigus.
Cirillo
N
N
Regional Center on Craniofacial Malformations-MRI, Second University of Naples, Naples, Italy. cirillo.sun@libero.it
Santoro
R
R
Lanza
M
M
Annese
P
P
Gombos
F
F
Lanza
A
A
D000051:Acantholysis / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D000328:Adult; D000818:Animals; D001323:Autoantibodies / Q000097:blood*; D051183:Desmoglein 1 / Q000276:immunology*; D006801:Humans; D007118:Immunoassay / Q000379:methods; D008297:Male; D051379:Mice; D009061:Mouth Mucosa / Q000473:pathology; D010392:Pemphigus / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D010641:Phenotype; D012867:Skin / Q000473:pathology
D001323:Autoantibodies; D051183:Desmoglein 1