Successful treatment of Rowell syndrome using oral cyclosporine A

To present and discuss successful treatment of Rowell syndrome with low dose orally administered cyclosporine in a male patient with refractory course of disease.

A 63-year-old male patient presented with a five-week history of widespread, severely pruritic erythematous skin changes that were first seen on the back. Within a few weeks, skin rashes extended to the whole body recessing the face. Because treatment with topical and orally administered corticosteroids was ineffective, we began an immunosuppressive therapy using cyclosporine. Treatment was initiated with an orally administered dosage of 100mg/day and subsequent dose reduction to 50mg/day after 14 days.

After four weeks of treatment, all clinical signs were resolved; only postinflammatory hyperpigmentation persisted for several weeks. Treatment-related side effects included arterial hypertension that could be controlled with antihypertensive drugs.

Primarily described in 1963, Rowell syndrome represents the combination of cutaneous lupus erythematosus and erythema multiforme in a single individual. Controversial discussion concerning the genuine character of the disease is still going on, and even the existence of this entity remains unclear. Several therapeutic regimes are known, including corticosteroids, methotrexate, or dapsone. To the best of our knowledge, this is the first report of successful treatment of Rowell syndrome with low doses of cyclosporine. Orally administered low-dose cyclosporine seems to be a potent alternative treatment option for patients with Rowell syndrome refractory to standard therapies.