Parry-Romberg Syndrome is a rare disorder characterized by:
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.
Epidemiology Disease Associations Pathogenesis Laboratory/Radiologic/
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Gross Appearance and Clinical Variants Histopathological Features and Variants Special Stains/
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EPIDEMIOLOGY CHARACTERIZATION SYNONYMS Parry-Romberg Syndrome
Progressive Facial Hemiatrophy
AGE RANGE-MEDIAN Majority before 20 years
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:
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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