Pemphigus Vulgaris

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Pemphigus vulgaris is a rare, potentially lethal autoimmune disease with intraepidermal bullae and extensive erosions in healthy appearing skin and mucous membranes. The diagnosis is made by direct immunofluorescence of a Hautbiopsates. For treatment, are sometimes used together with other immunosuppressive therapies, corticosteroids.

Most of pemphigus vulgaris occurs in middle-aged patients, where men and women are equally affected. Rare cases have been reported in children. A variant of paraneoplastic pemphigus may occur in patients with benign or malignant tumors. Most commonly, this variant occurs in patients with non-Hodgkin’s lymphoma.

Pemphigus vulgaris is a rare, potentially lethal autoimmune disease with intraepidermal bullae and extensive erosions in healthy appearing skin and mucous membranes. The diagnosis is made by direct immunofluorescence of a Hautbiopsates. For treatment, are sometimes used together with other immunosuppressive therapies, corticosteroids. Most of pemphigus vulgaris occurs in middle-aged patients, where men and women are equally affected. Rare cases have been reported in children. A variant of paraneoplastic pemphigus may occur in patients with benign or malignant tumors. Most commonly, this variant occurs in patients with non-Hodgkin’s lymphoma. Pemphigus vulgaris is characterized by IgG autoantibodies directed against the calcium-dependent cadherin and Desmoglein 1 desmoglein 3. These transmembrane glycoproteins affect cell-cell adhesion and signal transduction between epidermal cells. Acantholysis (loss of intercellular adhesion) results either from a direct inhibition of the function of the desmogleins by binding of autoantibodies or the fact that autoantibodies cause signal transduction through blistering and a down-regulation of cell-cell adhesion. These autoantibodies are present in the active phase of the disease in the serum as well as in the skin. This affects all areas of stratified squamous, including the mucosal surfaces. Symptoms and complaints Sagging bubbles that are the primary lesions of pemphigus vulgaris, cause widespread and painful erosions on the skin, oral and other erosion. About half of patients have oral erosions, which break up and remain as chronic, painful lesions for variable periods of time. Often oral lesions occur before the skin is affected. Dysphagia and poor oral intake are common, because the lesions can also occur in the upper esophagus. The cutaneous blisters typically occur on normal appearing skin, break open and leave a sore spot with crusting. A pruritus is usually absent. Erosions are prone to infection. For infestation of large areas of the body significant electrolyte and fluid losses are possible. Pemphigus vulgaris (flaccid bullae) Figure provided by Thomas Habif, M.D. var model = {thumbnailUrl: ‘/-/media/manual/professional/images/pemphigus_vulgaris_high_de.jpg?la=de&thn=0&mw=350’ imageUrl: ‘/-/media/manual/professional/images/pemphigus_vulgaris_high_de.jpg?la = en & thn = 0 ‘, title:’ pemphigus vulgaris (flaccid bullae) ‘description:’ u003Ca id = “v37894572 ” class = “”anchor “” u003e u003c / a u003e u003cdiv class = “” para “” u003e u003cp u003eDie primary lesions in pemphigus vulgaris are flaccid bullae u003c / p u003e u003c / div u003e. ‘credits’ figure provided by Thomas Habif

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