Abstract
BACKGROUND: Sclerodermic skin diseases can cause severe morbidity and disability. UVA-1 has shown to be an effective therapy for sclerodermic skin diseases. However, the period of remission in these patients is not clear. In this study, the effect and remission period of UVA-1 phototherapy in various sclerotic skin diseases is described using a semiquantitative clinical score combined with the durometer score as an objective apparatus to measure the hardness of the skin. OBJECTIVE: Our purpose was to determine the effectiveness of UVA-1 phototherapy and the duration of remission in sclerodermic skin diseases. METHODS: In this prospective study, 10 patients with various sclerodermic skin diseases were treated with UVA-1 phototherapy. The durometer was used to observe the hardness of the skin. Hardness of the skin was measured by one investigator at 10 locations, distributed evenly on the representative sclerotic skin. Each spot was measured three times, and the average of each of these measurements was summed to give the total durometer score. Durometer scores were recorded weekly until the final treatment date and 4 weeks after treatment. Clinical scores were carried out at the end date of the treatment using a 6-point scale semiquantitative score. Long-term effects were evaluated up to 29-46 months. RESULTS: The patients were treated with UVA-1 in a cumulative dose of 1286 +/- 58.8 (SEM) J/cm(2) (range, 846-1470 J/cm(2)) divided over five times a week for 4 weeks. In all patients studied, the sclerotic skin lesions were markedly softer after UVA-1 treatment. All durometer scores improved highly significant during the first 3 weeks of treatment and borderline significant during the last week of treatment. There was no significant improvement between the end of UVA-1 phototherapy and 1 month after ending the therapy (P > 0.05). All patients noted improvement of the semiquantitative clinical score during treatment. Clinical improvement was associated with improvement of the durometer score (95% confidence interval). With a follow-up until 46 months, the remission period was stable up to 26 months in six patients. The duration of sclerodermic skin diseases before start of treatment did not influence improvement in the clinical or durometer score. One patient had an acute side effect of minimal erythema. No other side effects, except tanning and fatigue, were noted. LIMITATIONS: This is an open-label uncontrolled study. CONCLUSION: UVA-1 is an effective treatment for sclerodermic skin diseases with a long period of remission and clinical improvement even in patients with a long history of a sclerotic skin disease. UVA-1 should be considered among the first approaches in the management of sclerotic skin diseases.
