Lupus profond et myélopathie

Abstract

We report a case of myelopathy during the course of lupus profundus that is unique to our knowledge. A 29-year-old woman had lupus profundus since 1999, initially associated with thrombopenia (28,000 platelets/mm3) treated with corticosteroids for 6 months. Several nodular eruptions occurred from 1999 to 2004. Antinuclear antibodies were positive at 1/320 to 1/640 and complement C4 fraction was low. While being treated with hydroxychloroquine for a recent flare-up of nodular lesions of lupus profundus, she complained of paraesthesia of the abdominal wall and of the upper and lower limbs, suggestive of a medullary lesion. MRI disclosed an image of acute myelitis at the level of the second cervical vertebra. High doses of corticosteroids were promptly administered intravenously (methylprednisolone bolus) followed by oral prednisone and hydroxychloroquine. Six months later the patient was free of neurologic or cutaneous symptoms. In the literature, systemic signs are rarely associated with lupus profundus and myelitis has never been reported. Lupus myelitis is a vascular and/or demyelinating and usually segmental lesion of the spinal cord. Neurologic symptoms are those of acute rather than chronic or recurrent transverse myelitis. The prognosis is poor with frequent and severe functional sequelae. Treatment is mainly based on high-dose systemic corticotherapy alone or combined with cyclophosphamide. Our patient responded favourably to early methylprednisolone bolus followed by oral corticosteroids and antimalarial treatment. Lupus profundus is classically of benign course with only cutaneous involvement, but it may sometimes be a sign of systemic lupus and in rare cases may be associated with severe complications.