Progressive hemifacial atrophy: a review.

Area of Science:

• Dermatology
• Neurology
• Rare Diseases

Background:

• Progressive Hemifacial Atrophy (PHA) is an acquired, unilateral facial distortion of unknown etiology, often considered a subtype of localized scleroderma.
• Historically debated, PHA is now generally accepted to exist on a spectrum with morphea en coup de sabre (ECDS), both forms of localized scleroderma.
• While trauma, autoimmunity, infection, and autonomic dysregulation are suggested etiologies, the pathogenesis remains unclear.

Purpose of the Study:

• To summarize the clinical findings, pathogenesis theories, diagnosis, clinical course, and proposed treatments for Progressive Hemifacial Atrophy (PHA).
• To provide a comprehensive overview of current understanding regarding this rare condition.
• To identify gaps in knowledge and the need for standardized guidelines.

Main Methods:

• Literature search using PubMed with terms including "idiopathic hemifacial atrophy," "Parry-Romberg syndrome," and "progressive hemifacial atrophy."
• Inclusion of original articles, reviews, case reports, and clinical guidelines.
• Focus on English language literature and available translations.

Main Results:

• PHA typically manifests in the first two decades but can present later.
• The condition progresses slowly over 2-20 years, eventually becoming quiescent.
• Common systemic associations include neurological manifestations like seizures and headaches.

Conclusions:

• Standardized guidelines for imaging, treatment, and follow-up for PHA are currently undefined.
• Further research is needed to elucidate the pathogenesis and establish evidence-based management strategies.
• PHA represents a complex condition requiring a multidisciplinary approach.