Clinical and Pathological Features of Linear Scleroderma with Central Nervous System Damage: A Case Report and Literature Review
Abstract：Objective: To explore the clinical manifestations, histopathological features, and treatment strategies of linear scleroderma (LS) with central nervous system damage. Methods: Clinical data and histopathological characteristics of a patient with LS and central nervous system damage were analyzed, and relevant literature on LS was systematically reviewed to summarize its clinical features and pathogenesis. Results: A female adolescent presented with skin hardening on the right forehead, a knife-like hair loss, and nasal wing atrophy. She underwent resection of the frontal brain lesion, corticosteroids, immunosuppressants, and antiepileptic drugs, with satisfactory results. Brain tissue biopsy showed glial cell and small vessel proliferation, vascular dilation, hyaline degeneration, and infiltration by lymphocytes and plasma cells. These findings suggest that vascular malformation combined with vasculitis may be an important pathogenic mechanism in this case. Conclusion: LS is often accompanied by ipsilateral neurological damage, and it is necessary to be vigilant against missed or misdiagnosis. Treatment recommendations include corticosteroids combined with methotrexate. For patients with severe central nervous system symptoms, excision of the lesion and early intervention are advised.
Keywords：Linear SclerodermaCentral Nervous System DamageClinical and Pathological Features
Publication Date：2025-04-20
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：3( 233-235 )