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Mid-dermal elastolysis
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Mid-dermal elastolysis

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Contributors: Masis Isikbay, Susan Burgin MD
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Synopsis

Mid-dermal elastolysis (MDE) is a rare acquired disorder characterized by the idiopathic loss of mid-dermal elastic fibers. MDE typically presents with asymptomatic, well-demarcated thin wrinkled plaques on the upper arms and trunk (type I). Preceding erythema is reported by a minority of patients and, even more rarely, urticarial plaques may be seen. Variant presentations of MDE include soft plaques with perifollicular protrusions with a peau d'orange appearance (type II) and reticular erythema (type III).

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.

Codes

ICD10CM:
L94.8 – Other specified localized connective tissue disorders

SNOMEDCT:
238821003 – Idiopathic mid-dermal elastolysis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Solar elastosis – yellowish papules, cobblestone plaques, and coarser wrinkling in sun-exposed areas
  • Anetoderma
  • 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

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Therapy

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References

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Last Updated: 07/20/2017
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Mid-dermal elastolysis
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Mid-dermal elastolysis : Peau d'orange, Smooth plaque, Trunk, Skin of back, Skin of upper arm
Clinical image of Mid-dermal elastolysis
A close-up of subtly pink plaques with a peau d'orange appearance.
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