En Coupe de Sabre – an Unusual case of Linear Scleroderma in Elderly female
Abstract
Linear Scleroderma is a rare autoimmune disorder, affecting approximately 3 per 100,000 people annually, distinguished by sclerotic changes in the skin and subcutaneous tissues. A specific subvariant, en coup de sabre, predominantly appears in children, with a higher incidence in females compared to males. This variant predominantly affects one side of the face, especially the frontoparietal region, manifesting as a linear streak within the dermatome. The affected area appears as a pigmented, shiny, and thickened patch of skin, resembling a sword strike due to the progressive atrophy of subcutaneous fat.
Fields of Study & Subjects
Systemic Sclerosis and Related Diseases
Autoimmune and Inflammatory Disorders
Dermatological and Skeletal Disorders
Publication details
- Journal / source
- World Allergy Organization Journal
- Volume
- 19
- Issue
- 2
- Pages
- 101287–101287
- Publication date
- 1 February 2026
- Language
- EN