Acquired cutis laxa. Primary generalized elastolysis

Cutis Laxa of the Face: A Case Report and Review of Literature

“…Several hypotheses have been proposed including excessive elastase activity, dysfunction of elastase inhibitors with abnormal copper metabolism, deficiency of copper, decreased production of tropoelastin, and immune mediated mechanisms [2,4].…”

“…Most of the cases are preceded by inflammatory conditions or malignancies. Type II (Marshall's syndrome) has been described in African children and North Americans and is characterized by localized elastolysis following inflammatory skin conditions [4].…”

Congenital Cutis laxa

“…Congenital cutis laxa is an extremely rare disorder of elastic tissue and includes heterogeneous groups of an autosomal dominant form, a severe autosomal recessive form and a X-linked recessive form [4][5][6][7], The son and his mother in case 1 and 2 were considered to be compatible with the autosomal dominant form, because of the characteristical clinical pictures and the family history.…”

“…or a rare. X-linked recessive form [4][5][6][7]. The clinical picture is characterized by inelastic, loose, and pendulous skin, which produces a wrinkled, prematurely aged, blood hound-like appearance.…”

Increased Fibroblast Elastase Activity in Acquired Cutis laxa

“…Acquired cutis laxa differs from inherited cutis laxa which is more common and can be transmitted as an autosomal dominant, autosomal recessive or X-linked recessive trait [1]. Acquired cutis laxa has been reported de novo or following inflammatory skin conditions such as Sweet’s syndrome [2, 3], drug reactions [4]or urticarial eruption [5, 6, 7, 8, 9]. It has also been described in association with neoplasia such as myeloma [10]or lymphoma [11].…”