Systemic sclerosis, localized morphea, en coup de sabre and aortic regurgitation: a rare association.

ABSTRACT

A 24-year-old male presented with Raynaud's phenomenon, digital infarcts, salt and pepper pigmentation and characteristic facies. There was gastrointestinal involvement clinically, endoscopically as well radiologically. In view of these findings and the demonstration of anti-nuclear antibodies with a homogeneous pattern of immunofluorescence and ScL-70 antibodies, he was diagnosed as a case of diffuse cutaneous systemic sclerosis. In addition, the patient had plaques of localized morphea, en coup de sabre with facial hemiatrophy on the left side, and mild aortic regurgitation (detected on echocardiography). The occurrence of these rare associations of localized plaques of morphea, en coup de sabre and aortic regurgitation in a single case of systemic sclerosis is quite an exceptional and interesting occurrence.