Neuroinflammatory Disease in Association with Morphea (Localized Scleroderma)

This chapter describes the case of a girl with a history of localized scleroderma (LS) who developed chronic and progressive seizures which were associated with focal brain lesions and chronic progression of the brain lesions. Localized scleroderma (LS) is an uncommon autoimmune condition of the skin that may be associated with head and facial lesions in one-fourth and neurological complications in one-fifth of those with head/facial lesions. Brain MRI may show atrophy or focal T2 hyperintensities on the side ipsilateral to the skin changes. Progression of brain lesions may occur insidiously. While no randomized control trials have been performed on immunotherapy for this condition, methotrexate, steroids, cyclophosphamide, mycophenolate mofetil, and azathioprine have been tried and may be effective.