Case of acquired cutis laxa with preceding urticarial eruption treated by diphenyl sulfone

Successful treatment of acquired cutis laxa with urticarial eruption by diphenyl sulfone

“…3 Six cases of acquired CL have featured urticarial eruption prior to the development of skin loosening and wrinkling, suggesting a pathogenic role of urticaria-associated inflammation in acquired CL. 4 Our case supports a role for inflammation in the pathogenesis of acquired CL. Moreover, the inflammation hypothesis is supported by development of acquired CL following other types of cutaneous inflammation.…”

“…CL is a rare skin disorder, with only 200 cases, including 50 acquired CL cases, reported in the literature 3 . Six cases of acquired CL have featured urticarial eruption prior to the development of skin loosening and wrinkling, suggesting a pathogenic role of urticaria‐associated inflammation in acquired CL 4 . Our case supports a role for inflammation in the pathogenesis of acquired CL.…”

“…Moreover, the inflammation hypothesis is supported by development of acquired CL following other types of cutaneous inflammation 5 . Although the pathological mechanisms remain unclear, excessive elastase activity associated with neutrophils and macrophages, and/or elastase inhibitor dysfunction, is likely to cause a loss of elasticity 4,6 . Mutations in the elastin (ELN), fibulin‐5 (FBLN5) or PYCR1 genes may also make elastic fibres susceptible to inflammation‐induced damage and biosynthetic disruption 7 …”

“…5 Although the pathological mechanisms remain unclear, excessive elastase activity associated with neutrophils and macrophages, and/or elastase inhibitor dysfunction, is likely to cause a loss of elasticity. 4,6 Mutations in the elastin (ELN), fibulin-5 (FBLN5) or PYCR1 genes may also make elastic fibres susceptible to inflammation-induced damage and biosynthetic disruption. 7 Although no satisfactory treatments are currently available for CL, our patient responded well to treatment with dapsone, possibly due to the inhibition of calcium influx, resulting in impairment of the calcium-dependent functions of neutrophils, such as oxidant production and protease secretion.…”

Chronic urticaria may not be as innocent as we think: A rare case of acquired cutis laxa following chronic urticaria

“…In the case of urticaria, infiltration of the neutrophils into the elastic fibers, increased elastase activity and dysfunction of elastase inhibitors, may cause fragmentation, thinning, and disorganization in ACL. [5][6][7] Since dapsone inhibits calcium-dependent activities of neutrophils, therapeutic management may be achieved by the use of dapsone. 8 By looking at the examples of successful treatment with dapsone in ACL patients, we chose to start with dapsone.…”