Parry-Romberg syndrome (progressive hemifacial atrophy)

Overview

Parry-Romberg syndrome, also called progressive hemifacial atrophy, is a rare acquired disorder characterized by slowly progressive shrinkage (atrophy) of the skin and soft tissues on one side of the face. The exact cause is unknown and the condition appears to occur sporadically, though some researchers suspect autoimmune or immune-mediated mechanisms may contribute due to its overlap with inflammatory and connective tissue disorders such as linear scleroderma.

Common Symptoms

Changes in skin color, facial bone or muscle loss, facial hair turning white and falling out, sunken-in appearance of the eyes and cheeks, shifting of the mouth and nose towards one side of the face, orthodontic issues, ophthalmologic problems, seizures, migraines, episodes of severe facial pain, facial bone loss, facial muscle atrophy, and retina and optic nerve issues.

Coexisting Diseases and Conditions

Alopecia, hyperpigmentation, vitiligo, seizures, and trigeminal neuralgia.

Risk Factors and Prevalence

Females are more likely to experience the condition than males. Those between the ages of 5 and 15 are most commonly affected. While no definitive cause is known, there are suspicions that Parry-Romberg syndrome is triggered by viral or bacterial infections, other autoimmune diseases, nervous system issues, brain or skull inflammation, and physical trauma.

Sources

  1. Article Sources
    1. Boston Children’s Hospital. (n.d.). Parry-Romberg syndrome: Boston Children’s Hospital. Boston Children’s Hospital.

    2. El-Kehdy, J., Abbas, O., & Rubeiz, N. (2012). A review OF PARRY-ROMBERG syndrome. Journal of the American Academy of Dermatology, 67(4), 769–784. https://doi.org/10.1016/j.jaad.2012.01.019

    3. U.S. Department of Health and Human Services. (2017, March 27). Parry-Romberg syndrome information page. National Institute of Neurological Disorders and Stroke. https://www.ninds.nih.gov/Disorders/All-Disorders/Parry-Romberg-Information-Page

    4. NORD (National Organization for Rare Disorders). (2020, April 6). Parry Romberg Syndrome. NORD (National Organization for Rare Disorders). https://rarediseases.org/rare-diseases/parry-romberg-syndrome/