OBJECTIVE To examine the frequency of antidesmoglein 1 (Dsg1) and antidesmoglein 3 (Dsg3) IgA autoantibodies in IgA pemphigus. DESIGN We developed an enzyme-linked immunosorbent assay against recombinant Dsg1 and Dsg3 to detect IgA autoantibodies. PATIENTS Twenty-two patients with IgA pemphigus were studied. Among them, 10 patients had subcorneal pustular dermatosis type, 9 patients had int...

Autoimmune bullous diseases are relatively uncommon and their treatment -- although generally similar -- may vary depending on the dermatologist. Within this group of diseases, the most common are pemphigus vulgaris and pemphigus foliaceus, bullous and mucosal pemphigoid, linear immunoglobulin A disease, and dermatitis herpetiformis. In recent years, the therapeutic arsenal has been extended by...

Autoimmune bullous diseases are relatively uncommon and their treatment—although generally similar—may vary depending on the dermatologist. Within this group of diseases, the most common are pemphigus vulgaris and pemphigus foliaceus, bullous and mucosal pemphigoid, linear immunoglobulin A disease, and dermatitis herpetiformis. In recent years, the therapeutic arsenal has been extended by new d...

Pemphigus herpetiformis (PH) is an uncommon variant of pemphigus that accounts for an estimated 6% to 7.2% of all cases of this skin disorder. The term pemphigus herpetiformis was coined by Jablonska et al. in 1975 to describe a entity that was clinically similar to dermatitis herpetiformis, showed acantholysis on biopsy, and responded to sulfapyridine. The authors considered that PH was a vari...

Desmogleins (Dsgs), cadherin-type cell adhesion molecules, are targeted in skin-blistering diseases such as pemphigus and staphylococcal scalded skin syndrome (SSSS). The role of Dsg4, a new isoform, was investigated in these diseases. Dsg4 was recognized by 30 (77%) of 39 pemphigus sera containing anti-Dsg1 IgG but not by 16 pemphigus sera containing no anti-Dsg1 IgG or by 34 normal control se...

The autoimmune blistering skin diseases pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are mainly caused by autoantibodies against desmosomal cadherins. In this study, we provide evidence that PV-immunoglobulin G (IgG) and PF-IgG induce skin blistering by interference with Rho A signaling. In vitro, pemphigus IgG caused typical hallmarks of pemphigus pathogenesis such as epidermal blister...

It has been suggested that anti-desmoglein autoantibody titers could be helpful in follow-up and therapeutic management of pemphigus patients. However, there is no consensus regarding the relationship between anti-desmoglein autoantibody titers and clinical activity of pemphigus.The aim of our study was to evaluate if clinical remission of pemphigus relates to the presence of anti-desmoglein au...