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Parry-Romberg syndrome in Adult
Other Resources UpToDate PubMed

Parry-Romberg syndrome in Adult

Contributors: Haya Raef MS, Arin Isenstein MD, David Dasher MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Parry-Romberg syndrome (PRS), also known as progressive facial hemiatrophy or idiopathic hemifacial atrophy, is a rare disorder that presents as slowly progressive atrophy of one half of the face. Skin and subcutaneous tissue atrophy may be accompanied by atrophy of muscle, cartilage, or bone. PRS commonly involves one or more of the trigeminal nerve branches. Severe disease is characterized by atrophy affecting the structures supplied by all 3 branches. Rare bilateral involvement and extrafacial involvement (such as the arm, leg, or trunk) has been reported.

The tongue and salivary glands may be affected. Associated neurologic and ophthalmic involvement may include migraine headaches, seizures, trigeminal neuralgia, enophthalmos, and eyelid dysfunction.

PRS typically develops in the first or second decade and is more common in females. It occurs spontaneously with no known preceding exposures or genetic predisposition, and is believed to be mediated by autoimmune factors. This syndrome may have an insidious onset and is slowly progressive, ultimately stabilizing after 2-20 years.

Codes

ICD10CM:
G51.8 – Other disorders of facial nerve

SNOMEDCT:
718224004 – Progressive hemifacial atrophy

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Bell palsy – Acute facial paralysis leading to facial asymmetry.
  • Linear morphea without PRS – Firm, indurated plaques with pigmentary change, with prominent dermal sclerosis on pathology.
  • Localized lipodystrophy – Atrophy of subcutaneous fat only.
  • Panniculitis

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed:07/20/2021
Last Updated:07/20/2021
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Parry-Romberg syndrome in Adult
Parry-Romberg syndrome : Enophthalmos, Muscle atrophy, Unilateral distribution, Teeth abnormalities
Clinical image of Parry-Romberg syndrome
Copyright © 2021 VisualDx®. All rights reserved.