Pemphigus is a group of autoimmune blistering disorders characterized by blister formation that is caused by loss of keratinocyte cell-to-cell adhesion in the epidermis due to circulating autoantibodies against desmosomal proteins [1]. The disease is extremely rare in pediatric age group. Pemphigus vulgaris (PV) is the most common form seen in children, as seen in adults. Although prevalence of...

IgA pemphigus is a newly characterized group of autoimmune intraepidermal blistering diseases presenting as vesiculopustular eruptions with a predilection in axillary and groin areas. Immunopathologically, IgA deposition in the intercellular space of the epidermis is present in all cases of IgA pemphigus by direct immunofluorescence. However, unlike pemphigus vulgaris, IgA pemphigus carries a r...

Pemphigus is an autoimmune blistering disease of the skin and mucous membranes. It is caused by autoantibodies directed against desmosomes, which are the principal adhesion structures between epidermal keratinocytes. Binding of autoantibodies leads to the destruction of desmosomes resulting in the loss of cell-cell adhesion (acantholysis) and epidermal blisters. The plasminogen activator system...

The aim of our study was to analyse a significance of tumour necrosis factor (TNF)-alpha promoter gene polymorphisms in relation to the HLA-DR locus in genetic predisposition to pemphigus. TNF-alpha gene polymorphisms in position -238 and -308 were identified using a modified polymerase chain reaction-restriction fragment length polymorphism method in 53 patients with pemphigus (38 with pemphig...

The usual outcome of pemphigus whitout treatment is death. The introduction of corticosteroids has greatly reduced the mortality of this condition, though a significant morbidity remains. The patients who are receiving high- dose steroids together with other immunosuppressive agents are at risk and may cause death through drug side effects and opportunitic infections. The descriptive study has ...

Pemphigus vulgaris pathogenesis had been considered well understood and was in danger of being “finished,” as was physics at the end of the 19th century. With the publication in this issue of a paper by Nguyen and colleagues (1), the understanding of pemphigus pathogenesis must undergo a major revision. This turning point brings with it the promise of a more complete picture, as well as new the...

ELISA: enzyme-linked immunosorbent assay IIF: indirect Immunofluorescence PNP: paraneoplastic pemphigus PV: pemphigus vulgaris INTRODUCTION Because of variability in clinical and histopathologic findings, the diagnosis of paraneoplastic pemphigus (PNP) frequently relies on the combination of direct immunofluorescence and indirect immunofluorescence (IIF). Here we present a unique case of PNP as...

BACKGROUND Immunosuppressive medications typically used to treat the immunobullous disorders pemphigus vulgaris, pemphigus foliaceous, and bullous pemphigoid can have serious adverse effects. The tetracycline family of antibiotic drugs has been shown to be effective in the treatment of these conditions with a more favorable side effect profile. Minocycline hydrochloride use has been associated ...

Immunosuppressive drugs are the milestone of treatment of autoimmune diseases, but they can lead to serious complications, including hepatitis B virus reactivation in HBV carriers as well as in patients with occult HBV infection (OBI). A 36-year-old man with OBI was diagnosed with pemphigus vulgaris. He was prescribed prednisolone and his hepatitis B surface antigen turned positive. Viral repli...

Pemphigus vulgaris (PV) is an uncommon, immune-mediated bullous dermatosis, which, during its active phase, has been associated with infertility. Pemphigus vulgaris during pregnancy is exceedingly rare-only 26 cases with immunopathological confirmation have been reported. The disease may be associated with adverse neonatal outcome, including prematurity and fetal death. Transient skin lesions m...