Benign chronic familial pemphigus (Hailey-Hailey disease) is a rare autosomal dominant blistering skin disorder characterized by suprabasal cell separation (acantholysis) of the epidermis. The Hailey brothers first described it in 1939. Hailey-Hailey disease usually appears in the third or fourth decade, although it can occur at any age. Heat, sweating and friction often exacerbates the disease...

Paraneoplastic pemphigus (PNP), a clinically and immunopathologically distinct mucocutaneous blistering dermatosis, is a severe form of autoimmune multiorgan syndrome generally associated with poor therapeutic outcome and high mortality. This IgG-mediated disease is initiated by an obvious or occult lymphoproliferative disorder in most cases. Clinically severe mucositis, and polymorphic blister...

Pemphigus vulgaris is a potentially fatal autoimmune mucocutaneous disease associated with production of IgG autoantibodies to desmoglein 3, a 130 kDa epidermal protein. To further characterize the epitope(s) of Pemphigus vulgaris antigen we established two humanhuman hybridomas by fusion of the peripheral blood mononuclear cells with a human and mouse heterohybridoma. These hybridomas designat...

A morbidly obese 60-year-old Hispanic woman with chronic obstructive pulmonary disease, renal failure and asthma but no history of skin disease was hospitalized for a putative urinary tract infection. She had developed a red pruritic painless rash 2 days after starting famotidine. She was allergic to ampicillin and had taken dexamethasone, prozac, percocet, diltiazem, theophylline, ciprofloxaci...

VOLUME 74, NOVEMBER 2004 285 Blister beetles (Figure) are the source for commercial preparations of cantharidin. Many blister beetle species exist, and many have not been studied extensively. Much of what we know about blister beetles is extrapolated from studies of a few species. In some blister beetles, such as Epicauta funebris, cantharidin has been identified in all 10 life stages and accum...

BACKGROUND Hailey-Hailey disease (HHD) is a rare, chronic and recurrent blistering disorder, which is characterized clinically by erosions occurring primarily in intertriginous regions, and histologically by suprabasal acantholysis. Oxidative stress plays a specific role in the pathogenesis of HHD, by regulating the expression of factors playing an important role in keratinocyte proliferation a...

Darier disease (DD) is an autosomal dominant inherited skin disorder, characterized by abnormal keratinization, loss of adhesion between epidermal cells, termed acantholysis, and the development of warty papules and plaques on the central trunk, forehead, scalp and flexures. These symptoms are often exacerbated by heat, sweating, sunburn and stress. Mutations in the ATP2A2 gene, encoding SERCA2...

KP: Koebner phenomenon PV: pemphigus vulgaris INTRODUCTION Pemphigus vulgaris (PV) is a rare autoimmune mucocutaneous blistering disease characterized by epidermal acantholysis, flaccid bullae, and erosive lesions in the skin and mucosa. The Koebner phenomenon (KP) is defined as the appearance of new, typical skin lesions on areas of injury in otherwise healthy skin. KP is described in several ...

Background. Pemphigus vulgaris is an autoimmune blistering disease affecting the mucous membrane and skin. In 50 to 70% of cases, the initial manifestations of pemphigus vulgaris are oral lesions which may be followed by skin lesions. But it is unusual for the disease to present with initial and solitary persistent lower lip lesions without progression to any other location. Main Observations. ...

Pemphigus foliaceus (PF) is an organ-specific autoimmune skin disease characterized by subcorneal epidermal cell detachment (acantholysis) and pathogenic autoantibodies against desmoglein 1. The mechanism responsible for pemphigus autoantibody-induced epidermal injury is not fully understood. In this study, we used the IgG passive transfer mouse model of PF to investigate the relevance of the a...