Background

Parry-Romberg Syndrome is a rare disorder characterized by:

Hemifacial atrophy
Distinctive changes of the eyes and hair
Neurological abnormalities including seizures and trigeminal neuralgia

The symptoms and signs become apparent during the first or early during the second decade of life, but rarely at birth. The majority of individuals with Parry-Romberg Syndrome experience symptoms before the age of 20 years.

The hemifacial atrophy typically progresses over approximately three to five years and then ceases.

OUTLINE

Epidemiology
Disease Associations
Pathogenesis
Laboratory/Radiologic/ Other Diagnostic Testing
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Special Stains/ Immunohistochemistry/ Electron Microscopy
Differential Diagnosis
Prognosis
Treatment
Commonly Used Terms
Internet Links

EPIDEMIOLOGY	CHARACTERIZATION
SYNONYMS	Parry-Romberg Syndrome Progressive Facial Hemiatrophy
AGE RANGE-MEDIAN	Majority before 20 years

 

GROSS APPEARANCE/ CLINICAL VARIANTS	CHARACTERIZATION
General
VARIANTS
Facial changes	Involve the tissues above the upper jaw (maxilla) or between the nose and the upper corner of the lip (nasolabial fold) and progress to involve the angle of the mouth, the areas around the eye, the brow, the ear, and/or the neck Progressive tissue wasting is limited to one half of the face, usually the left side Affected areas may demonstrate shrinkage and atrophy of tissues beneath the skin, subcutaneous fat, and underlying cartilage, muscle, and bone.
Skin	Skin overlying affected areas: Hyperpigmentation Vitiligo
Oral cavity	Atrophy of half of the upper lip and tongue as well as abnormal exposure, delayed eruption, or wasting of the roots of certain teeth on the affected side
Hair	Appear on the affected side: Whitening (blanching) of the hair as well as abnormal bald patches on the scalp and loss of eyelashes and the middle (median) portion of the eyebrows (alopecia)
Neurological	Severe headaches that last for extended periods of time and may be accompanied by visual abnormalities, nausea, and vomiting (migraines) Facial pain (trigeminal neuralgia) Periods of uncontrolled electrical disturbances in the brain (seizures) with rapid spasms of a muscle group that spread to adjacent muscles (contralateral Jacksonian epilepsy)

 

PROGNOSIS AND TREATMENT	CHARACTERIZATION
Prognostic Factors
Survival	Range and severity of associated symptoms and findings may vary

Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.

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Last Updated 3/08/2001

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