Pediatric Patients With Progressive Hemifacial Atrophy: Clinical Features, Course, and Treatment

Area of Science:

• Dermatology
• Pediatrics
• Rare Diseases

Background:

• Progressive hemifacial atrophy (PHA), also known as Parry-Romberg syndrome, is a rare disorder.
• It is characterized by progressive atrophy of facial tissues and is considered part of the morphea spectrum.
• PHA typically manifests in childhood, presenting diagnostic challenges.

Purpose of the Study:

• To investigate the clinical characteristics, treatment, and outcomes of pediatric patients diagnosed with PHA.
• To highlight the diagnostic delay and management complexities associated with this rare condition.

Main Methods:

• A longitudinal, ambispective, observational study was conducted.
• The study included 8 pediatric patients diagnosed with PHA.
• Data collected included age at onset and diagnosis, clinical presentation, extracutaneous involvement, treatments received, and surgical interventions.

Main Results:

• The mean age at symptom onset was 4.5 years, and at diagnosis was 8.2 years, indicating a significant diagnostic delay.
• All patients exhibited cutaneous findings, and 7 out of 8 had associated maxillofacial, ophthalmological, or neurological abnormalities.
• Patients received an average of 2.2 immunosuppressive regimens for a mean duration of 35.2 months, and 4 underwent fat grafting.

Conclusions:

• Pediatric PHA patients experience considerable diagnostic delays.
• Management requires long-term, multidisciplinary care involving systemic immunosuppressive therapy and surgical interventions.
• Frequent extracutaneous abnormalities necessitate comprehensive management strategies.