Localized scleroderma, also called morphea, is an inflammatory skin condition causing excess collagen deposition and thickened skin. Parry-Romberg Syndrome is one subtype characterized by linear facial involvement. Rarely, neurologic symptoms manifest as seizures, neuropathy, CNS vasculitis, and white matter abnormalities on MRI. Prior case reports of CNS involvement are associated with T2 hyperintense lesions, predominantly subcortical and juxtacortical, ipsilateral to the cutaneous lesions and often localized to one brain area. Multiple sclerosis (MS) is a primary CNS demyelinating condition, most frequently with a relapsing remitting presentation. It is characterized by white matter abnormalities on MRI, usually with periventricular, juxtacortical, cortical or brainstem and spinal cord lesions. We present a 41-year-old African American female previously diagnosed with MS and started on ofatumumab. She presented in hopes of switching therapies due to injection fatigue. On exam, she exhibited left facial discoloration and hemifacial atrophy, progressively worsening for years. She was referred to a dermatologist and subsequently diagnosed with morphea via skin biopsy. MRI showed bihemispheric periventricular, subcortical, and juxtacortical demyelinating lesions and global atrophy. She also had cervical and thoracic spinal cord involvement. Imaging was more suggestive of MS than CNS morphea, and the concomitant diagnosis of both conditions was made. Given that none of the disease modifying therapies approved for MS are compatible with treatment of morphea, this presented a challenging scenario. The patient was continued on a B-cell therapy for MS and started on monthly methylprednisolone infusions for morphea, with clinical stability to date.
We present a unique diagnostic dilemma of concomitant presentation of MS and morphea versus CNS involvement of morphea. Ultimately, the distinct imaging features of both disorders on MRI guided this patient’s diagnosis and treatment.