Relapsing-remitting lesions in a woman with progressive hemifacial atrophy and chronic hepatitis B virus infection: A case report.

Abstract

Introduction:Progressive hemifacial atrophy (PHA) is a rare disorder characterized by unilateral facial atrophy affecting the skin, subcutaneous tissue, and fat, muscle, and osteocartilagenous structures creating a sunken hemiface appearance.Etiopathogenesis of PHA is poorly understood; no definitive treatment is currently available.Clinical Findings:We report a 41-year-old woman with PHA who showed an uncharacteristic “relapsing–remitting” evolution of brain lesions and was seropositive for hepatitis B virus (HBV). She presented with a history of recurrent tonic-clonic seizures. Magnetic resonance imaging (MRI) showed progressive atrophy and multiple white matter lesions in the left side of the brain. Interestingly, the serial MRI examination (4 MRI scans over a period of 9 years) showed a “relapsing–remitting” pattern of brain lesions akin to that observed in a subtype of multiple sclerosis. Autoimmune-related investigations revealed increased serum levels of immunoglobulin (Ig) G, anti-nuclear antibody (ANA), and γ-IgG. Infection is considered as one of the possible causes of PHA. However, the association of peripheral infection such as HBV infection with PHA has not been reported.Conclusion:Our experience with this case suggests that PHA may have a relapsing–remitting disease course. Autoimmune inflammatory response to chronic HBV infection may have triggered the relapse in this case. This case underlines a novel etiopathogenetic mechanism of PHA.