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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.


Commonly Used Terms

Basic Principles of Disease
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Commonly Used Terms
This is a glossary of terms often found in a pathology report.

Diagnostic Process
Learn how a pathologist makes a diagnosis using a microscope

Surgical Pathology Report
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Special Stains
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How Accurate is My Report?
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Got Path?
Recent teaching cases and lectures presented in conferences


Internet Links

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Last Updated 3/08/2001

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