Cutis Laxa

Hallmarks of Cutis Laxa is a loose skin that hangs down in folds. The diagnosis is made clinically. There is no specific treatment, but sometimes plastic surgery can help.

Cutis Laxa can be inherited or acquired. There are 4 hereditary forms: an autosomal dominant form, an X-linked recessive form 2 and autosomal recessive forms. The autosomal recessive forms are somewhat more common. One of the recessive forms can cause potentially fatal cardiac, respiratory and gastrointestinal complications. The other hereditary forms are relatively benign.

Hallmarks of Cutis Laxa is a loose skin that hangs down in folds. The diagnosis is made clinically. There is no specific treatment, but sometimes plastic surgery can help. Cutis Laxa can be inherited or acquired. There are 4 hereditary forms: an autosomal dominant form, an X-linked recessive form 2 and autosomal recessive forms. The autosomal recessive forms are somewhat more common. One of the recessive forms can cause potentially fatal cardiac, respiratory and gastrointestinal complications. The other hereditary forms are relatively benign. Rarely infants acquire a Cutis laxa for a febrile infection or after exposure to certain drugs (eg. B. allergic reaction to penicillin, fetal exposure to penicillamine). In children and adults, a cutis laxa develops after a severe illness with fever, polyserositis or erythema multiforme. In adults, it can be insidious or in conjunction with a number of diseases, especially Plasmazellendyskrasien develop (plasma cell diseases at a glance). The underlying defect in these acquired cases is not known, but a fragmented elastin is present in all forms. Pathophysiology Cutis laxa is caused by abnormal elastin metabolism and results in decreased skin elasticity. The exact cause is unknown, except in congenital cases where an underlying genetic defect (z. B. in the ELN, FBLN4-, FBLN5-, ATP6V0A2- or ATP7A genes) can be identified. Various factors such. B. copper deficiency, Elastinmenge and morphology as well as elastases and elastase inhibitors are involved in the abnormal elastin. Symptoms and complaints It occurs in all places where the skin is usually loose and hangs in folds, but especially in the face. The congenital forms of the laxity of the skin at birth may be present or develop later. Affected children have a sorrowful, wrinkled face and a hooked nose. The benign autosomal recessive form also causes a geiszige disability and joint hyperextensibility. Hernias and diverticula of the gastrointestinal tract are common. A progressive pulmonary emphysema may be reflected in severe affected patients in a pulmonary heart disease. Bronchiectasis, heart failure and aortic aneurysms may also occur. Cutis Laxa © Springer Science + Business Media var model = {thumbnailUrl: ‘/-/media/manual/professional/images/517-cutis-laxa-s110-springer-high_de.jpg?la=de&thn=0&mw=350’ imageUrl ‘/-/media/manual/professional/images/517-cutis-laxa-s110-springer-high_de.jpg?la=de&thn=0’, title: ‘cutis laxa’ description: ‘ u003Ca id = ” v37897647 “class = ” anchor “” u003e u003c / a u003e u003cdiv class = “” para “” u003e u003cp u003eDieses photo shows loose skin folds in a child with dermatochalasis. u003c / p u003e u003c / div u003e ‘credits’ © Springer Science + Business Media’

Health Life Media Team

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