Pediatric Patients With Progressive Hemifacial Atrophy

Progressive hemifacial atrophy (PHA) or Parry-Romberg syndrome is a rare, insidiously progressive disorder that typically presents in childhood and is considered part of the morphea spectrum. This longitudinal, ambispective, observational study included 8 pediatric patients (4 female) diagnosed with PHA, with a mean age at symptom onset of 4.5 ± 2 years and at diagnosis of 8.2 ± 2.9 years. All 8 patients presented superficial and deep cutaneous findings (dyspigmentation, dermal and subcutaneous atrophy, and facial asymmetry); 7 had associated extracutaneous abnormalities (maxillofacial, ophthalmological, and neurological), all received systemic immunosuppressive therapy (average of 2.2 ± 1 regimens for a mean duration of 35.2 ± 29.3 months), and 4 had subsequent fat grafting procedures. In conclusion, pediatric patients with PHA suffer an important diagnostic delay, require long-term treatment with multiple immunosuppressive drugs and surgical interventions, as well as multidisciplinary management of frequent associated extracutaneous abnormalities.

Abstract from PubMed