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Parry Romberg disease - Skin
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Parry Romberg disease - Skin

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Contributors: Arin Isenstein MD, David Dasher MD, Jeffrey D. Bernhard MD, Sarah Stein MD, Karen Wiss MD, Sheila Galbraith MD, Craig N. Burkhart MD, Dean Morrell MD, Lynn Garfunkel MD, Nancy Esterly MD
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Synopsis

Parry-Romberg syndrome, also known as progressive facial hemiatrophy or idiopathic hemifacial atrophy, is thought to be a rare form of linear morphea presenting as slowly progressive deterioration of one half of the face. Hemiatrophy includes loss of subcutaneous tissue, muscle, and bone. Rare bilateral involvement 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.

Parry-Romberg syndrome 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. This syndrome is slowly progressive and can have an insidious onset. Initially, the asymmetry is subtle, noted first by the patient and caregivers only. Autoantibodies (antinuclear antibodies [ANA]) are detected in up to 50% of patients with this syndrome.

Codes

ICD10CM:
G51.8 – Other disorders of facial nerve

SNOMEDCT:
95834000 – Facial hemiatrophy

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Bell's palsy – acute facial paralysis leading to facial asymmetry
  • Linear morphea – 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 Updated: 02/12/2015
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Parry Romberg disease - Skin
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Parry Romberg disease : Atrophy, Cheek, Erythema, Muscle atrophy, Unilateral
Clinical image of Parry Romberg disease
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