Acquired cutis laxa

Acquired cutis laxa (ACL), also known as acquired elastolysis, is a rare connective tissue disorder characterized by loose, redundant, and inelastic skin that gives the affected individual a prematurely aged appearance. Unlike inherited forms of cutis laxa, ACL develops during a person's lifetime rather than being present from birth. The condition results from the destruction or loss of elastic fibers in the skin and potentially other organs. ACL can occur at any age but most commonly presents in adulthood. It may develop following an inflammatory skin condition (such as urticaria, erythema multiforme, or Sweet syndrome), after a severe illness, following drug exposure (such as penicillin or isoniazid), or in association with other systemic diseases including lymphoma, multiple myeloma, or other hematologic disorders. In some cases, no identifiable trigger is found. The skin is the most prominently affected organ, with patients developing pendulous folds of skin, particularly on the face, neck, and trunk, leading to a characteristic aged or hound-dog facial appearance. However, ACL can also involve internal organs. Pulmonary involvement may lead to emphysema and respiratory insufficiency, while cardiovascular complications can include aortic aneurysms, peripheral arterial disease, and cardiac valve abnormalities. Gastrointestinal involvement with diverticula and hernias may also occur. The severity of systemic involvement varies considerably among patients. There is no definitive cure for acquired cutis laxa. Treatment is primarily directed at managing the underlying cause when one is identified, such as treating an associated malignancy or discontinuing an offending medication. Reconstructive or cosmetic surgery may be performed to address the redundant skin, particularly on the face, although results can be variable and skin laxity may recur. Patients with pulmonary or cardiovascular involvement require monitoring and appropriate management of these complications. Dapsone and other anti-inflammatory agents have been tried in some cases with variable success, particularly when an inflammatory process is ongoing.

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