Juvenile localized scleroderma with CNS involvement: a clinical case of successful treatment

Boyko Ya.Ye.^1,2, Pavliuk М.А.¹, Kozachuk О.М.¹, Boyko M.T.¹

• ¹Clinical center of childrens’ healthcare, Lviv
• ²Danylo Halytskyi Lviv National Medical University, Lviv

Summary. Juvenile localized scleroderma (JLS) includes a group of disorders whose manifestations are limited to lesions of the skin and subcutaneous tissues and, with minor exceptions, the nervous system, and do not affect internal organs. The most common neurological manifestations of JLS include headache, as well as seizures (often resistant to anticonvulsant drugs), Rasmussen’s encephalitis, peripheral neuropathy, impaired intellectual development, and neuropsychiatric symptoms. The article presents the case of a patient with JLS who was diagnosed with juvenile localized scleroderma en coup de sabre with resistant epilepsy and uveitis. The patient received anticonvulsant therapy without the desired effect. The appointment of complex immunosuppressive therapy (tocilizumab, methotrexate, and mycophenolate mofetil) made it possible to achieve control over seizures and eliminate cognitive disorders. Further studies are needed to optimize the therapeutic strategy and determine the indications for the use of biologics in JLS, particularly in the presence of CNS involvement.