" /> Elastosis perforans serpiginosa - CISMeF





Preferred Label : Elastosis perforans serpiginosa;

ICD-11 definition : An uncommon dermatosis due to transepidermal extrusion of degenerate dermal elastin. It is particularly associated with Down syndrome but may also be seen in association with Ehlers-Danlos, Marfan, and Rothmund-Thomson syndromes, osteogenesis imperfecta and pseudoxanthoma elasticum. Rarely it may be associated with penicillamine therapy. It presents as multiple small keratotic papules arranged in an annular or serpiginous pattern and typically is located on the sides of the neck, the face or the arms.;

ICD-11 synonym : Perforating elastoma;

ICD-11 inclusion : Perforating elastoma; Miescher's elastoma;

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An uncommon dermatosis due to transepidermal extrusion of degenerate dermal elastin. It is particularly associated with Down syndrome but may also be seen in association with Ehlers-Danlos, Marfan, and Rothmund-Thomson syndromes, osteogenesis imperfecta and pseudoxanthoma elasticum. Rarely it may be associated with penicillamine therapy. It presents as multiple small keratotic papules arranged in an annular or serpiginous pattern and typically is located on the sides of the neck, the face or the arms.

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24/05/2025


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