A 26-year-old woman presented with a 5-month history of painful oral erosions and a 1-month history of widespread flaccid vesicles over her face and torso. A biopsy specimen of a skin lesion revealed suprabasal acantholysis. The diagnosis of pemphigus vulgaris (PV) was confirmed by a positive intercellular (IC) antibody titer. The patient was treated with prednisolone (30-45 mg/d) combined with...

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...

UNLABELLED A RARE DISEASE: Paraneoplastic pemphigus is an rare autoimmune bullous skin disease recently recognized. About 50 cases have been reported since its first description in 1990. CLINICAL MANIFESTATIONS Clinical signs are polymorphous resembling the cutaneomucosal manifestations of pemphigus vulgar (skin and mucosa erosions, fragile interdermal bullae), pemphigoid (urticaria, distende...

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. ...

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...

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...

Pemphigus herpetiformis (PH) is an uncommon variant of pemphigus that accounts for an estimated 6% to 7.2% of all cases of this skin disorder. The term pemphigus herpetiformis was coined by Jablonska et al. in 1975 to describe a entity that was clinically similar to dermatitis herpetiformis, showed acantholysis on biopsy, and responded to sulfapyridine. The authors considered that PH was a vari...

Dear Editor, We report the case of a Japanese man with Dari-er's disease (DD) that affected the esophagus as well as the skin. A 49-year-old man, who was diagnosed with DD 19 years earlier, visited us again in October 2008 because his skin lesions had exacerbated. Physical examination revealed reddish-brown crusted fol-licular papules mostly coalesced to produce irregularly shaped warty plaques...

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...