Cutis laxa

Cutis laxa [1] or pachydermatocele[2] is a group of rare connective tissue disorders in which the skin becomes inelastic and hangs loosely in folds.

[citation needed] In contrast, acquired cutis laxa often has a triggering event such as urticaria, drugs (such as penicillin) or neoplasms.

[15] Acquired cutis laxa may also be immunologically mediated, as it can involve dermal deposit of immunoglobulins and it can occur with autoimmune diseases.

[15] It has also been considered that mutations in elastin (ELN) and fibulin-5 (FBLN5) genes can increase susceptibility of elastic fibres to inflammatory degradation in acquired cutis laxa.

[15] Acquired cutis laxa has also been seen in conjunction with a number of conditions including: rheumatoid arthritis,[16] systemic lupus erythematosus,[17] celiac disease,[5] and monoclonal gammopathies.

[19] Urticarial skin fibroblasts have shown a 2- to 3- fold increase in elastase activity in a patient with acquired cutis laxa.