Parry-Romberg Syndrome

Parry-Romberg Syndrome

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What is Parry-Romberg Syndrome?

Parry-Romberg syndrome (PRS) is a rare condition in which there is progressive gradual shrinkage and degeneration of the skin, muscles and soft tissues of one side of the face. The cartilage and bony structures of the face may also be affected.

It is also known as hemifacial atrophy (HFA), progressive hemifacial atrophy or Romberg syndrome.

The children suffering from Parry-Romberg syndrome are normal at birth and the symptoms usually start between five and fifteen years. The symptoms progress slowly and the disease stabilizes between two to ten years. However, the deformities suffered due to tissue degeneration are usually permanent.

Parry-Romberg syndrome was first described by Caleb Parry in 1825 and Moritz Romberg in 1846. PRS is usually limited to the face, but may also affect the limbs unilaterally.

What are the Causes of Parry-Romberg Syndrome?

The exact causes of Parry-Romberg syndrome are still unknown. However certain possible factors that influence PRS are:

  • Trauma to the face or neck
  • Bacterial or viral infections
  • Autoimmune disorders such as systemic lupus erythematosus (SLE), rheumatoid arthritis, and inflammatory bowel disease (IBD)
  • Inflammation of the brain
  • More common in girls
  • Scleroderma
  • Intracranial vascular dysplasia
  • Abnormal development of the nervous system
  • Neurodegeneration

References:

  1. Parry-Romberg Syndrome - National Organization for Rare Disorders (NORD) - (https://rarediseases.org/rare-diseases/parry-romberg-syndrome/)
  2. Madasamy R Jayanandan M, Adhavan UR, Gopalakrishnan S, Mahendra L. Parry Romberg Syndrome: A Case Report and Discussion. J Oral Maxillofac Pathol. 2012; 16(3): 406-10. - (https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3519218/)
  3. Ortega VG, Sastoque D. New and Successful Technique for the Management of Parry-Romberg Syndromes Soft Tissue Atrophy. J Craniofac Surg. 2015; 26(6): e507-10. - (https://www.ncbi.nlm.nih.gov/pubmed/26335318)
  4. Sargunar B, Ebenezer V, Balakrishnan R, Priya S. Parry Romberg Syndrome-A Review of Treatment Options. Biomed Pharmacol J. 2014; 7(1): 207-11. - (http://biomedpharmajournal.org/vol7no1/parry-romberg-syndrome-a-review-of-treatment-options/)
  5. Wong M, Phillips CD, Hagiwara M, D.R. Shatzkes DR. Parry Romberg Syndrome: 7 Cases and Literature Review. Am J Neuroradiol. 2015; 36(7): 1355-61. - (http://www.ajnr.org/content/36/7/1355)

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