Effective Oral Psoralen Plus Ultraviolet A Therapy for Digital Ulcers with Revascularization in Systemic Sclerosis

Abstract

Systemic sclerosis (SSc) is a heterogeneous disorder characterized by excessive fibrosis and microvascular damage to the skin and various internal organs, including the lungs, kidneys, oesophagus and heart. Raynaud’s phenomenon is an almost universal symptom of SSc; 95% of patients are affected. Digital ulcers are observed frequently, especially in intractable cases with severe Raynaud’s phenomenon, and there is considerable morbidity, with gangrene and amputations (1). To date, there is no promising treatment for severe cases of SSc that are complicated by digital ulcers. Apart from immunosuppressive drugs, administration of oral psoralen plus ultraviolet A (PUVA) therapy has been used in patients with SSc with progressive skin sclerosis complications, with some improvement (2). We report here the first case of successful oral PUVA therapy in a patient with SSc complicated by progressive skin sclerosis and digital ulcers.