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Elastosis perforans serpiginosa in Adult
Other Resources UpToDate PubMed

Elastosis perforans serpiginosa in Adult

Contributors: Jeffrey M. Cohen MD, Vivian Wong MD, PhD, Belinda Tan MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

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.

Codes

ICD10CM:
L87.2 – Elastosis perforans serpiginosa

SNOMEDCT:
49428008 – Elastosis perforans serpiginosa

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References

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Last Reviewed:07/20/2021
Last Updated:01/16/2022
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Elastosis perforans serpiginosa in Adult
A medical illustration showing key findings of Elastosis perforans serpiginosa : Crust, Neck, Symmetric extremities distribution, Firm papules, Arms
Clinical image of Elastosis perforans serpiginosa - imageId=35519. Click to open in gallery.  caption: 'Serpiginous, scaling, and crusted, erythematous plaques on the posterior neck.'
Serpiginous, scaling, and crusted, erythematous plaques on the posterior neck.
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