Most reported patients are women of Northern European descent between the ages of 30 and 50 years. Men with MDE have a slightly older age of onset. Systemic involvement is not a feature.
The pathogenesis of this condition has not been fully elucidated; however, purported pathomechanisms include ultraviolet light-induced elastic fiber degeneration, defects in the synthesis of elastic fibers, and autoimmune destruction thereof.
L94.8 – Other specified localized connective tissue disorders
238821003 – Idiopathic mid-dermal elastolysis
Differential Diagnosis & Pitfalls
- Solar elastosis – yellowish papules, cobblestone plaques, and coarser wrinkling in sun-exposed areas
- Perifollicular elastolysis – white perifollicular papules with loss of elastic fibers limited to the perifollicular location
- Pseudoxanthoma elasticum (PXE)
- Fibroelastolytic papulosis – Term that encompasses white fibrous papulosis of the neck and PXE-like papillary dermal elastolysis. White or yellowish papules on the neck and torso display diminished elastin and fibrillin and/or thickened collagen bundles in the papillary and reticular dermis.
- Cutis laxa