E60 Localized scleroderma and venous thrombosis: an uncommon association in a case report

Abstract

Abstract Introduction Morphea or localized scleroderma is a rare paediatric inflammatory disease. It causes sclerosis of the skin and subcutaneous tissues. The condition does not have serious systemic implications, except in rare cases. However, it can cause functional and aesthetic sequelae. There is no truly effective consensus treatment, hence the importance of correctly assessing the extent and severity of the disease before any therapeutic approach. Objective Through the case of a girl presenting with linear scleroderma, revealed at the exploration of a superficial venous thrombosis; we report the diagnostic and therapeutic difficulty. Results Hadjer, a 14 years old girl, with no particular personal or family history, presented with thrombosis of the great saphenous vein. Thrombophilia work-up and anti-phospholipid antibodies were normal. She was treated by stripping; then she was lost to follow-up. One year later, hadjer’s clinical examination revealed thickened, inflamed skin on the left lower limb, extending from the dorsal surface of the ankle to the upper third of the thigh along its inner surface, suggesting linear scleroderma in the inflammatory phase. There was also a varicose vein sequelae of the thrombosis. The patient had no Raynaud's phenomenon or systemic damage. The skin biopsy confirmed the diagnosis. The search for multi-visceral involvement was non-contributory: capillaroscopy, chest X-ray, cardiac ultrasound, ophthalmological examination, liver, kidney and haematological tests. Muscle enzymes and magnetic resonance imaging for deep involvement were also normal. The autoimmune assessment revealed the positivity of Antinuclear Antibodies. The patient benefited from oral corticosteroid therapy and physiotherapy. After three months of partial remission, methotrexate was started. Discussion and conclusion Morphea is a rare autoimmune condition in paediatrics. A correlation between antinuclear antibody positivity and the severity of Morphea has been suggested. Certain risk factors have been incriminated. The linear form is by far the most frequent. The presence of venous thrombosis suggests the probable association of other autoimmune diseases, hence the interest of a rigorous follow-up, to watch for other clinical signs. The treatment and prognosis of morphea depend on the clinical form and the time to diagnosis; functional and aesthetic sequelae can be observed in the deep forms which require treatment with corticoids and immune modulators. Ethics this work is ethically approved.