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Kraus V, Lawson EF, Scheven EV Atypical cases of scleroderma En Coup de Sabre. J Child Neurol. 2012; 29:698-703
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‘En coup de sabre’ or morphea, is a linear scleroderma that typically affects the frontoparietal region of patients resulting in a band-like scar that looks like a sword cut across the face. This case report describes a 13-year-old male patient with a history of ‘en coup de sabre’ referred to a dental department because of facial and dental involvement. To the authors' knowledge this has only previously been described on one occasion. The clinical presentation, clinical findings and a brief discussion of management, including the complicating effects of ‘en coup de sabre’ are discussed.
CPD/Clinical Relevance: By describing the facial and dental features of this rare condition to the dental community, the authors wish to increase awareness of the condition.
Article
The term scleroderma encompasses a rare group of fibrosing disorders that affect the skin and underlying tissues.1,2 In recent years, the classification of scleroderma has changed and the subtypes now described include localized scleroderma (or morphea), limited cutaneous systemic sclerosis, diffuse cutaneous systemic sclerosis and systemic sclerosis sine scleroderma.1 Localized scleroderma differs from systemic sclerosis in the absence of systemic involvement and is reported to have an incidence of 0.4 to 2.7 per 100,000 and is more commonly seen in females.1,2,3
‘En coup de sabre’ skin lesions are located in the head region and are characteristically fibrotic hyperpigmented plaques, like areas which are permanently hairless that resemble the stroke of a sword.2,3,4,5 These lesions can affect underlying bone, eye tissue and brain parenchyma, although rarely cross the midline.2,5 Lesions are benign in nature and typically start to develop at a young age in patients, progress over a number of years and then remain stationary into adult life.5 ‘En coup de sabre’ scleroderma mainly affects children, with over 90% of patients presenting aged between 2 and 14 years of age.2,3 Lesions are classically found in the frontoparietal region, involve the scalp and often can extend into ocular and nasal tissue in a vertical vector pattern.5
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