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.;
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.