P148 Clinical profile and treatment outcomes in en coup de sabre: experience from a tertiary centre

Abstract

En coup de sabre (ECDS) is a rare variant of linear localized scleroderma characterized by a linear, indurated and atrophic lesion involving the frontoparietal scalp and face. ECDS may be associated with neurological, ophthalmological and craniofacial involvement, contributing to significant morbidity. Early diagnosis and systemic immunosuppression are crucial to prevent disease progression and permanent sequelae. The aim of this study was to retrospectively evaluate the clinicodemographic profiles and treatment outcomes of patients with ECDS managed at a tertiary care centre. This retrospective study included 10 patients diagnosed with ECDS and registered between January 2023 and December 2024. Data extracted from medical records included clinicodemographic details; sites of involvement; laboratory investigations including antinuclear antibody (ANA) and brain magnetic resonance imaging (MRI) findings; treatment details and follow-up outcomes. All patients were treated with oral corticosteroids for 6 months along with weekly methotrexate for a duration of 1–1.5 years. The mean age of patients was 18.4 years (range 8–30), with a female-to-male ratio of 6 : 4. Age at disease onset ranged from 3 to 21 years, while disease duration at presentation varied from 0.5 to 19 years. Scalp and nasal involvement was observed in eight and five patients, respectively, and all patients had scalp and eyebrow alopecia. ANAs were negative in all cases. Brain MRI abnormalities were detected in seven patients. Three patients were lost to follow-up. Among the remaining patients, disease control was achieved within 1–2 months of treatment initiation, and sustained remission was observed at follow-up ranging from 1 to 3 years from baseline. ECDS demonstrates a heterogeneous clinical spectrum with frequent neurological involvement. Systemic therapy with oral corticosteroids and methotrexate was effective in achieving rapid disease control and sustained remission. Early diagnosis, routine neuroimaging and long-term follow-up are essential for optimal management of this rare condition.