Pemphigus vulgaris (PV) is a potentially lethal mucocutaneous blistering disease characterized by cell-cell detachment within the stratified epithelium (acantholysis) caused by IgG autoantibodies. Intravenous immunoglobulin (IVIg) therapy effectively treats PV, but the mechanism is not fully understood. To further understand acantholysis and the efficacy of IVIg, we measured effects of IgG frac...

Linear acantholytic dermatoses are a spectrum of cutaneous disorders that form a subset of linear dermatoses with distinct clinical features and histopathologically show acantholysis. The lesions may be zosteriform or follow the lines of Blaschko. This report describes a four-year-old boy who, on a follow up of two years, exhibited a relapsing acantholytic dermatosis along the lines of Blaschko...

Patients with keratotic lesions distributed in a unilateral, linear, zosteriform or localized pattern and revealing histopathologic features of dyskeratotic acantholysis have been reported previously. There is some controversy in the appropriate nosologic classification of this entity. Some authors consider it as a localized form of Darier’s disease while others place it as a variant of e...

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...

Subcorneal pustular dermatosis (SCPD, also known as Sneddon-Wilkinson disease) is a rare, benign, chronic, sterile pustular eruption which usually develops in middle-age or elderly women; it is rarely seen in childhood and adolescence. The primary lesions are pea-sized pustules classically described as half-pustular, half-clear flaccid blisters. Histologically the most important feature is a su...

Pemphigus is a group of human autoimmune blistering diseases of the skin in which autoantibodies to desmosome cadherins induce loss of cell-cell adhesion (acantholysis). In addition to steric hindrance and activation of intracellular signaling, apoptosis has been suggested to contribute to the mechanism by which pathogenic IgG induces acantholysis. We review the current literature examining the...

BACKGROUND Pemphigus vulgaris (PV) is an autoimmune, IgG autoantibody-mediated disease of skin and mucosa leading to progressive blistering and nonhealing erosions. Patients develop autoantibodies to adhesion molecules mediating intercellular adhesion and to keratinocyte cholinergic receptors regulating cell adhesion. OBSERVATIONS To determine whether a cholinergic agonist can abolish PV IgG-...