Blistering diseases are alarming skin conditions where blister formation occurs in various ways and cannot be differentiated clinically. For confirmation of diagnosis, along with routine histological examination, immunofluorescence study is essential. Tzanck smear may be used as a rapid diagnostic tool. We included here 34 cases of different blistering lesions. Other than routine Hematoxylin an...

Autoimmune phenomena occur in healthy individuals, but when self-tolerance fails, the autoimmune response may result in specific pathology. According to Witebsky's postulates, one of the criteria in diagnosing a disease as autoimmune is the reproduction of the disease in experimental animals by the passive transfer of autoantibodies. For epidermolysis bullosa acquisita (EBA), a prototypic organ...

Blister formation in skin and mucous membranes results from a loss of cell-cell or cell-matrix adhesion and is a common outcome of pathological events in a variety of conditions, including autoimmune and genetic diseases, viral and bacterial infections, or injury by physical and chemical factors. Autoantibodies against structural components maintaining cell-cell and cell-matrix adhesion induce ...

Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease characterized by deposition of autoantibodies at the basement membrane zone. In an experimental BP model in mice, the subepidermal blistering is mediated by antibodies directed against the hemidesmosomal protein BP180 (collagen XVII, BPAG2), and depends on complement activation and neutrophil infiltration. Gelatinase B is ...

Kindler syndrome is a rare autosomal recessive disorder associated with skin fragility. It is characterized by blistering in infancy, photosensitivity and progressive poikiloderma. The syndrome involves the skin and mucous membrane with radiological changes. The genetic defect has been identified on the short arm of chromosome 20. This report describes an 18-year-old patient with classical feat...

BACKGROUND The dermatoscopic features of facial lentigo maligna (LM), facial lentigo maligna melanoma (LMM) and acral lentiginous melanoma (ALM) have been well described. This is the first description of the dermatoscopic appearance of a clinical series of cutaneous non-facial non-acral lentiginous growth pattern melanomas. OBJECTIVE To describe the dermatoscopic features of a series of cutan...

Genetically engineered mice have been essential tools for elucidating the pathological mechanisms underlying human diseases. In the case of diseases caused by impaired desmosome function, mouse models have helped to establish causal links between mutations and disease phenotypes. This review focuses on mice that lack the desmosomal cadherins desmoglein 3 or desmocollin 3 in stratified epithelia...

W. Nishie , M.D., Ph.D. • H. Shimizu , M.D., Ph.D. (*) Department of Dermatology , Hokkaido University Graduate School of Medicine , N15W7, Kita-ku , Sapporo 003-0835 , Japan e-mail: [email protected] Abstract Bullous pemphigoid (BP) is the most common autoimmune blistering disorder. BP autoantibodies target two hemidesmosomal components, collagen XVII (COL17) and BP230, with autoimmuni...

A 5-year-old boy presented with a history of dry scaly skin. He had been born at term, with no collodion membrane or erythroderma noted at delivery. Skin changes were noted soon after birth, with widespread dryness and occasional blistering, mainly affecting the toes. Previous treatment with emollients and topical corticosteroids had not resulted in improvement. There was no family history of d...

OBJECTIVE To investigate the clinical, genetic, and laboratory features of 26 patients with Kindler syndrome. DESIGN Case series of patients recruited when they were seen at outpatient consultations in the Department of Dermatology at the Changuinola Hospital in Bocas del Toro, Panama, between May 1986 and December 1990. SETTING Clinical history, physical examination, and laboratory studies...