Elastosis perforans serpiginosa in Adult
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Synopsis
EPS may arise in childhood or early adulthood. There is a slight predilection for males. EPS is associated with Ehlers-Danlos syndrome type IV, Marfan syndrome, acrogeria, scleroderma, Rothman-Thompson syndrome (poikiloderma congenitale), cutis laxa, berry aneurysms, osteogenesis imperfecta, pseudoxanthoma elasticum, or Down syndrome in approximately 40% of cases. Up to 25%-50% of cases of EPS are related to D-penicillamine use in some series. Generally, EPS arises after extended periods of use of D-penicillamine. EPS may occur without an underlying disease or drug cause.
Codes
L87.2 – Elastosis perforans serpiginosa
SNOMEDCT:
49428008 – Elastosis perforans serpiginosa
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Last Updated:01/16/2022