EPIDERMOLYSIS BULLOSA DYSTROPHICA PDF

Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed. Dystrophic epidermolysis bullosa DEB is a form of inherited epidermolysis bullosa EB characterized by cutaneous and mucosal fragility resulting in blisters and superficial ulcerations that develop below the lamina densa of the cutaneous basement membrane and that heal with significant scarring and milia formation. The clinical picture varies widely, ranging from mild to severe.

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DermNet provides Google Translate, a free machine translation service. Note that this may not provide an exact translation in all languages. Epidermolysis bullosa EB is a group of inherited diseases that are characterised by blistering lesions on the skin and mucous membranes.

These may occur anywhere on the body but most commonly appear at sites of friction and minor trauma such as the feet and hands. In some subtypes, blisters may also occur on internal organs, such as the oesophagus , stomach and respiratory tract, without any apparent friction.

Dystrophic epidermolysis bullosa DEB is characterised by the site of blister formation in the lamina densa within the basement membrane zone and the upper dermis. It causes generalised blistering of the skin and internal mucous membranes and leads to scar formation. The latter is the more severe form. In the dominant subtypes of epidermolysis bullosa, where an informative family tree is known, it is often acceptable for clinical diagnosis to be made by a specialist dermatologist based on the presenting signs.

See smartphone apps to check your skin. DermNet NZ does not provide an online consultation service. If you have any concerns with your skin or its treatment, see a dermatologist for advice. Dystrophic epidermolysis bullosa — codes and concepts open. Genetic disorder, Systemic disorder. Genetics and molecular biology of dystrophic epidermolysis bullosa, Clinical features of dystrophic epidermolysis bullosa, Treatment of dystrophic epidermolysis bullosa, Prognosis of dystropic epidermolysis bullosa.

Multicentre consensus recommendations for skin care in inherited epidermolysis bullosa. Orphanet J Rare Dis. Inherited epidermolysis bullosa: updated recommendations on diagnosis and classification. J Am Acad Dermatol. Epub Mar Generalised blistering present at birth Blistering becomes localised to hands, feet, elbow or knees as the child grows older and in response to friction Small white spots called milia are often present at healed but scarred sites Bart syndrome : aplasia cutis , lesions in the mouth, and abnormal nails due to abnormal type 7 collagen in anchoring fibrils May also get blistering of the oesophagus.

SCC look and behave differently in EB from in unaffected individuals. They are found on covered sites and grow rapidly.

They present as ulcerated or keratotic nodules and plaques.

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Epidermolysis bullosa dystrophica

DermNet provides Google Translate, a free machine translation service. Note that this may not provide an exact translation in all languages. Epidermolysis bullosa EB is a group of inherited diseases that are characterised by blistering lesions on the skin and mucous membranes. These may occur anywhere on the body but most commonly appear at sites of friction and minor trauma such as the feet and hands. In some subtypes, blisters may also occur on internal organs, such as the oesophagus , stomach and respiratory tract, without any apparent friction. Dystrophic epidermolysis bullosa DEB is characterised by the site of blister formation in the lamina densa within the basement membrane zone and the upper dermis.

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Dystrophic epidermolysis bullosa

Dystrophic epidermolysis bullosa is one of the major forms of a group of conditions called epidermolysis bullosa. Epidermolysis bullosa cause the skin to be very fragile and to blister easily. Blisters and skin erosions form in response to minor injury or friction, such as rubbing or scratching. The signs and symptoms of dystrophic epidermolysis bullosa vary widely among affected individuals. In mild cases, blistering may primarily affect the hands, feet, knees, and elbows. Severe cases of this condition involve widespread blistering that can lead to vision loss, scarring, and other serious medical problems.

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