Parry-Romberg Syndrome: A Report of a Rare Case and a Comprehensive Review

Parry-Romberg syndrome (PRS), also recognized as progressive hemifacial atrophy (PHA), is a rare medical condition affecting the dermis, subcutaneous tissue, and occasionally underlying anatomical structures such as muscles and bones. While the etiology of this condition remains incompletely elucida...

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Bibliographic Details
Published inCurēus (Palo Alto, CA) Vol. 16; no. 8; p. e67345
Main Authors Marappan, Harikrishnan, Am, Raja
Format Journal Article
LanguageEnglish
Published 20.08.2024
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Summary:Parry-Romberg syndrome (PRS), also recognized as progressive hemifacial atrophy (PHA), is a rare medical condition affecting the dermis, subcutaneous tissue, and occasionally underlying anatomical structures such as muscles and bones. While the etiology of this condition remains incompletely elucidated, it has been hypothesized that trauma, autoimmunity, infection, and autonomic dysregulation may constitute potential contributory factors. Typically, the onset of symptoms occurs within the initial two decades of life, though instances of late-onset PRS manifesting in the sixth and seventh decades of life have also been documented. The disorder is distinguished by a gradual progression over two to 20 years, ultimately culminating in stabilization. The local manifestations of PRS are accompanied by systemic symptoms. Common neurological complications include seizures and headaches. Due to the rarity of PRS, there are no established guidelines for imaging, treatment, and follow-up. Therefore, management is tailored to each case, with treatment options primarily addressing symptoms.Parry-Romberg syndrome (PRS), also recognized as progressive hemifacial atrophy (PHA), is a rare medical condition affecting the dermis, subcutaneous tissue, and occasionally underlying anatomical structures such as muscles and bones. While the etiology of this condition remains incompletely elucidated, it has been hypothesized that trauma, autoimmunity, infection, and autonomic dysregulation may constitute potential contributory factors. Typically, the onset of symptoms occurs within the initial two decades of life, though instances of late-onset PRS manifesting in the sixth and seventh decades of life have also been documented. The disorder is distinguished by a gradual progression over two to 20 years, ultimately culminating in stabilization. The local manifestations of PRS are accompanied by systemic symptoms. Common neurological complications include seizures and headaches. Due to the rarity of PRS, there are no established guidelines for imaging, treatment, and follow-up. Therefore, management is tailored to each case, with treatment options primarily addressing symptoms.
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ISSN:2168-8184
2168-8184
DOI:10.7759/cureus.67345