It is characterized clinicallyby flaccid bullae leading to erosions; histologically by the formation of intraepidermal blisters resulting from acantholysis, and immunopathologically by the presence of in vivo bound and circulating IgG autoantibodies against keratinocyte cell-surface components, the desmosomal desmoglein 3
and desmoglein 1.1,2 Indian patients of pemphigus are also known to present at a younger age.1 The disease used to be fatal in 60-90% cases before the advent of corticosteroids.1,3 After the introduction of corticosteroid therapy in the 1950s, the mortality rate of patients with pemphigus declined from 90% to 24%.4,5 But high dose steroids given for a prolonged period of time led to many adverse effects which also contributed to mortality.
Mucocutaneous manifestations are the result of intercellular autoantibodies mainly of the IgG type working against desmosomal antigens desmoglein 3
(Dsg3) in the mucosal dominant type and additionally against desmoglein 1 (Dsg1) in the mucocutaneous type (20).
The grafts were assessed for the presence of progenitor cells, and the predominant phenotype (>50%) consisted of small cells positive for [DELTA]Np63, CK14, and ABCG2 and negative for CK3/12 and desmoglein 3
. They reported a clinical success rate of 67% defined as persistent continuous epithelial surface and a significant decrease of corneal neovascularization .
demonstrated that by ELISA techniques, in the case of pemphigus, salivary desmoglein 1 and desmoglein 3
had sensitivities of 70% and 94%, respectively, in the diagnosis of this dermatological condition .
Setterfield, "Serum and salivary IgG and IgA antibodies to desmoglein 3
in mucosal pemphigus vulgaris," British Journal of Dermatology, vol.
The currently known target antigens are desmoglein 1 (Dsg1), desmoglein 3
(Dsg3), and plakin protein, which act as adhesion molecules between keratinocytes in the epidermis.
Stanley, "Pemphigus vulgaris antigen (desmoglein 3
) is localized in the lower epidermis, the site of blister formation in patients," The Journal of Investigative Dermatology, vol.
Another CAM, Desmoglein 3
(DSG3), is a desmosomal cadherin that mediates cell-cell adhesion via desmosomes .
Autoantibodies are produced against desmoglein 3
or both desmoglein 1 and desmoglein 3
in pemphigus vulgaris.
(132) However, over-expression of certain adhesion proteins, such as claudin 1 (133) and desmoglein 3
, (134) can enhance invasion.
was previously identified as a tissue-specific antigen in MRL-lpr mice .