Dystrophic epidermolysis bullosa (DEB) is a severe skin fragility disorder associated with trauma-induced blistering, progressive soft tissue scarring, and increased risk of skin cancer. DEB is caused by mutations in type VII collagen. In this study, we describe the generation of a collagen VII hypomorphic mouse that serves as an immunocompetent animal model for DEB. These mice expressed collag...

Epidermolysis bullosa acquisita is a subepidermal blistering disease associated with tissue-bound and circulating autoantibodies against type VII collagen, a major constituent of the dermal-epidermal junction. The passive transfer of Abs against type VII collagen into mice induces a subepidermal blistering disease dependent upon activation of terminal complement components. To further dissect t...

Stevens-Johnson syndrome is a rare but serious medical condition, which classified within the blistering diseases of skin and mucous membranes. It characterized by severe generalized immune-mediated inflammatory reaction, predominantly affecting membranes, such as eyes, mouth genitalia.

Background Epidermolysis bullosa simplex (EBS) is the most common type of EB, a group rare genodermatoses. Affected individuals suffer from skin blistering and report high disease burden. In some EBS subtypes, plantar keratoderma (PK) has been described. Objectives This study investigated presence correlation PK with body mass index, pain mobility in EBS. Methods Individuals (n = 157) genetical...

Linear IgA/IgG bullous dermatosis (LAGBD) is an auto-immune blistering disease characterized by the local accumulation of IgA- and IgG-class anti-basement membrane autoantibodies. It typically presents as a generalized pruritic vesiculobullous eruption. No cases of localized LAGBD have yet been reported. We report a case of a 78-year-old man with LAGBD localized to the perianal area. The patien...

Bullous systemic lupus erythematosus is one of the rare autoantibody mediated skin manifestation of systemic lupus erythematosus (SLE) demonstrating subepidermal blistering with neutrophilic infiltrate histologically. We present a case of a 40-year-old Hispanic female who presented with a several months' history of multiple blistering pruritic skin lesions involving the face and trunk, a photos...

Maintenance of an intact epidermis depends on secure adhesion between adjacent keratinocytes and between basal keratinocytes and underlying epidermal basement membrane. The major adhesion units that achieve this are the hemidesmosomes and desmosomes but when these structures are disrupted, for example by gene mutations or autoantibodies, the resilience of the epidermis is lost and blisters deve...

The importance of desmosomal cell adhesion to human health is evidenced by the autoimmune disease pemphigus vulgaris (PV), in which autoantibodies against the extracellular domain of the desmosomal cadherin desmoglein 3 cause potentially fatal blistering of the skin and mucous membranes. Tucker et al. describe how enhanced expression of a desmosomal cytoplasmic plaque protein, plakophilin-1, pr...

INTRODUCTION Paraneoplastic pemphigus is an autoimmune disease associated with an underlying tumor. Several cases have been reported as unusual pemphigus vulgaris, erythema multiforme, or paraneoplastic bullous disease, but the incidence of the condition is unknown. Response to treatment is generally poor, with significant morbidity and mortality. This condition is clinically characterized by s...

Pemphigus vulgaris is an autoimmune blistering disease affecting the mucous membrane and skin. Ulcers, vesicles, bulla, erosions are the common manifestations of the disease. It is uncommon to find multiple pustular lesions in the oral cavity. Here, we report the first case of multiple pustules involving the lateral borders of tongue, buccal mucosa, hard palate, soft palate, vestibule and the g...