Elastosis perforans serpiginosa in Child
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Synopsis

Elastosis perforans serpiginosa (EPS) is a disorder of elastic fibers characterized by transepidermal elimination of abnormal elastic fibers. It is 1 of 4 classic perforating dermatoses that are now grouped under the umbrella term "acquired perforating dermatosis"; this grouping also includes reactive perforating collagenosis, perforating folliculitis, and Kyrle disease.
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.
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
ICD10CM:
L87.2 – Elastosis perforans serpiginosa
SNOMEDCT:
49428008 – Elastosis perforans serpiginosa
L87.2 – Elastosis perforans serpiginosa
SNOMEDCT:
49428008 – Elastosis perforans serpiginosa
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Drug Reaction Data
Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.
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Last Reviewed:07/20/2021
Last Updated:01/16/2022
Last Updated:01/16/2022