A 43 year old woman. Chronic Cutaneous Lupus Erythematosus (CCLE) onset at the age of 25 with initial lesions on the scalp, face, trunk, and hands (generalized CCLE) that progressively extended regardless of prolonged treatment with antimalarials ( 18 yrs) and topical steroid therapy.
At the age of 39 she started complaining of arthralgia, constitutional symptoms, vasculitic lesions on the hands and feet. Laboratory investigations revealed antinuclear, anti-nRNP and anti-Sm autoantibodies indicating a late evolution towards Systemic Lupus Erythematosus.
She was therefore treated with prednisone (0.5 mg/Kg/d) with adequate control of the constitutional symptoms and other complaints. However no significant improvement in the scalp lesions was observed.
Recent reports suggest that only a minority of CCLE patients progress towards systemic disease (less than 5% during adequate follow-up). The extent, distribution and evolution of the cutaneous lesions are regarded as relevant "prognostic factors" and there is general agreement that patients with long-standing, treatment resistant, generalized CCLE are more likely to develop SLE.