En Coup De Sabre - Localized Scleroderma
Overview:
En coup de sabre localized scleroderma is a rare type of scleroderma, a connective tissue disorder characterized by hardening and thickening of the skin. It is called en coup de sabre because it typically affects only one area of the face, giving the appearance of being struck by a saber.
Cause:
The exact cause of en coup de sabre is unknown, but it is thought to be related to autoimmune dysfunction, in which the immune system attacks healthy cells and tissues.
Symptoms:
Symptoms may include hardening and thickening of the skin on the affected area of the face, as well as atrophy (thinning) of the skin and underlying tissues, and loss of hair in the affected area.
Source: https://commons.wikimedia.org/wiki/File:Frontal_linear_scleroderma_1.JPEG
License: Gambichler et al., CC BY-SA 2.0 <https://creativecommons.org/licenses/by-sa/2.0>, via Wikimedia Commons
Diagnosis:
Diagnosis of en coup de sabre typically involves a physical examination and skin biopsy.
Treatment:
Treatment of en coup de sabre may include medications such as corticosteroids or immunosuppressants to reduce inflammation and suppress the immune system, as well as physical therapy to maintain range of motion and prevent contractures. In severe cases, surgical intervention may be necessary to correct deformities or remove excess skin.
Prognosis / Outlook:
The prognosis for en coup de sabre is generally good, although it can be associated with significant cosmetic deformities and functional limitations. Proper treatment can help to minimize these effects and improve the overall outcome.