Hemifacial Atrophy, Progressive

Alternative Names

  • HFA
  • Parry-Romberg Syndrome
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WHO-ICD-10 version:2010

Diseases of the nervous system

Nerve, nerve root and plexus disorders

OMIM Number

141300

Mode of Inheritance

Autosomal dominant; Isolated cases

Description

Parry-Romberg syndrome was first described by Parry in 1825 and then by Romberg in 1846. It is a rare, acquired disorder characterized by slow and progressive atrophy of soft tissues of half the face. Usually the left side of the face is affected and in some cases, the facial muscles, cartilages, as well as, the bones are involved. Other symptoms may develop in some patients including neurological abnormalities, optic nerve damage, bone loss, alopecia, and pigmentation irregularities.

The degree of atrophy can vary widely, ranging from mild, barely perceptible changes to significant asymmetry. The disorder is more common in females than in males. The onset of the Parry-Romberg syndrome is usually between the ages of 5 and 15 years. To date, there is no cure and there are no treatments that can stop the progression of Parry-Romberg syndrome.

Molecular Genetics

The exact cause of Parry-Romberg syndrome is unknown; cases appear to occur sporadically for unknown reasons.

Epidemiology in the Arab World

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Other Reports

Libya

Singh et al. (1985) reported what could be the first case of Romberg's syndrome from Libya in a young boy.

Saudi Arabia

Al-Khenaizan and Al-Watban (2005) reported a 28-year-old Saudi female with Parry-Romberg syndrome, who presented with facial asymmetry.  At the age of 21 years, she noticed shrinkage in the skin of the right side of her face, associated with scalp hair loss on the same side.  She also suffered from anxiety, depression, and rheumatic valvular heart disease.  Atrophy and loss of the subcutaneous fat with brownish hyperpigmentation and prominent veins was presented in her right submental region.  Though no affective treatment was given, her disease had not been progressing.

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