Relapsing–remitting lesions in a woman with progressive hemifacial atrophy and chronic hepatitis B virus infection

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.

Progressive Hemifacial Atrophy and Linear Scleroderma En Coup de Sabre: A Spectrum of the Same Disease?

“…Progressive hemifacial atrophy can be provoked by viral infections ( 43 ). Zhang and colleagues reported a 41-year-old woman with PHA who showed an uncharacteristic “relapsing-remitting” evolution of brain lesions and chronic HBV infection may have triggered the relapse in this case ( 113 ).…”

A Case Report of Parry–Romberg Syndrome Misdiagnosed as Multiple Sclerosis

“…Some patients with PRS exhibit a "relapsing-remitting" course, which is a common feature of MS (18). Our patient suffered from recurrent paroxysmal weakness of the right hand, but the symptom occurred for about 1 h almost every day for 3 years.…”

“…In addition to reports implicating autoimmunity, there are indications that PRS can be evoked by infection. Zhang et al (18) described a case of PRS that was apparently triggered by peripheral hepatitis B virus. Asanad (17) described a patient who developed PRS after herpes zoster infection, with post-herpetic neuralgia in the distribution of the ophthalmic division of the trigeminal nerve.…”

Parry–Romberg Syndrome with Uhthoff’s Phenomena: A Spectrum of Autoimmune Disease?

“…In addition, the patient's PRS followed a herpetic infection in the ophthalmic division of the trigeminal nerve with subsequent facial atrophy in the corresponding region. MRI revealed cerebral and periventricular white matter lesions, a hallmark pattern of MS. Intriguingly, the current patient presented with heat-induced horizontal diplopia on right lateral gaze with radiographic evidence of INO involving the MLF, a clinical presentation also consistent with MS. A recent report by Zhang et al showed that PRS may have a disease course similar to the “relapsing-remitting” subtype of MS as triggered by a chronic hepatitis B virus infection [14]. Infiltration of the central nervous system by immune cells and microbial infections likely serve as immunological triggers for demyelination in MS [15].…”

“…As the most common ocular manifestation of PRS, enophthalmos develops from retro-orbital fat or bone atrophy [14]. Patients with PRS may present with diplopia secondary to restricted ocular motility resulting from either ocular motor nerve dysfunction, extraocular muscle degeneration, or fibrosis [15].…”