Abstract
Localized scleroderma is traditionally considered to be limited to the skin, subcutaneous tissue, underlying bone, and in the craniofacial subtype, also nervous system involvement. However, recent studies have also described other systemic manifestations in these patients. Despite many reports of neurological involvement in patients with the craniofacial linear localized scleroderma, it is extremely rare in patients with the other subtypes of localized scleroderma. Here, we report an extraordinary case of localized scleroderma en plaque (classic morphea), located to the upper trunk and neck, associated with neurological manifestations presented as seizures. Magnetic resonance imaging of the brain showed focal lesions on the contralateral side to the skin involvement. This case is extremely relevant not only due to its rarity, but also because it supports the idea that the pathogenesis of the localized scleroderma is related to a systemic autoimmune process.
Key points
• Localized scleroderma is known to be related with systemic symptoms.
• To the best of our knowledge, this is the first case report of an exclusive plaque morphea with neurological involvement.
• This case provides support to the concept that the pathogenesis of the disease is a systemic autoimmune process.
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Rosario, C., Garelick, D., Greenberg, G. et al. Plaque morphea with neurological involvement—an extraordinary uncommon presentation. Clin Rheumatol 34, 597–601 (2015). https://doi.org/10.1007/s10067-013-2458-1
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DOI: https://doi.org/10.1007/s10067-013-2458-1