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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.

For more information, see OMIM.

Codes

ICD10CM:
L87.2 – Elastosis perforans serpiginosa

SNOMEDCT:
49428008 – Elastosis perforans serpiginosa

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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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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References

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Last Reviewed:07/20/2021
Last Updated:07/28/2021
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Elastosis perforans serpiginosa in Adult
Elastosis perforans serpiginosa : Crust, Neck, Symmetric extremities distribution, Firm papules, Arms
Clinical image of Elastosis perforans serpiginosa
Serpiginous, scaling, and crusted, erythematous plaques on the posterior neck.
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