Parry-Romberg Syndrome With Localized Scleroderma: A Report of Two Pediatric Cases From Oman

Parry-Romberg syndrome (PRS) is a rare, acquired neurocutaneous disorder characterized by progressive hemifacial atrophy, primarily affecting the skin, soft tissue, and underlying bone. The etiology remains unclear, though autoimmune, inflammatory, and genetic factors have been implicated. PRS frequently coexists with localized scleroderma (en coup de sabre), leading to diagnostic and therapeutic challenges. Here, we present two pediatric cases from tertiary care hospitals in Oman, emphasizing clinical presentation, diagnostic findings, and treatment response. The first patient, a 10-year-old male, presented with progressive left-sided facial atrophy since age seven, associated with green discoloration beneath the left eye. Skin biopsy confirmed epidermal atrophy with lipodystrophy. MRI revealed asymmetric lateral ventricles with mild left facial atrophy. He was treated with IV methylprednisolone, followed by oral prednisolone and methotrexate, resulting in improved soft tissue bulk. The second patient, a nine-year-old female, developed right-sided facial atrophy at age five, with a hyperpigmented macule on the mandible. MRI was normal, while histopathology showed epidermal atrophy with increased basal pigmentation and dermal fibrosis. She initially received methotrexate but developed transaminitis, necessitating a switch to mycophenolate mofetil. Disease progression was halted following immunosuppressive therapy. PRS predominantly affects females and typically presents in the first two decades of life. Our cases align with reported literature, with both patients developing symptoms in early childhood. Neurological involvement, commonly reported in PRS, was absent in our patients. MRI findings were inconsistent with the literature, as one patient exhibited mild atrophy while the other had a normal scan. The presence of dental anomalies, including malocclusion and caries, underscores the multisystemic impact of PRS. Current treatment strategies focus on halting progression using corticosteroids and immunosuppressants, as demonstrated by favorable outcomes in our cases. PRS remains a diagnostic challenge due to its variable presentation and uncertain pathogenesis. Early recognition and immunosuppressive therapy can mitigate disease progression and improve outcomes. Further studies are needed to elucidate long-term prognostic factors and optimal management strategies.