Ultraviolet A1 phototherapy beyond morphea: experience in 83 patients.

BACKGROUND/PURPOSE: Ultraviolet A1 (UVA1) phototherapy has been used for over 15 years in the United States, primarily for the treatment of localized sclerosis and various sclerosing disorders. The objective was to describe use of UVA1 for dermatoses beyond localized sclerosis at two academic institutions. METHODS: Data from 83 patients treated with low- (20-40 J/cm(2) ), medium- (>40-80 J/cm(2) ), and high- (>80-120 J/cm(2) ) dose UVA1 phototherapy was retrospectively analyzed. The mean individual treatment dose (J/cm(2) ), the mean number of sessions, and the mean total dose (J/cm(2) ) were evaluated. Effectiveness was assessed by reviewing clinical examination notes from office visits. RESULTS: Good therapeutic efficacy was seen in patients with systemic sclerosis (SS, 16 patients), graft-versus-host disease (GVHD, 25 patients), and nephrogenic systemic fibrosis (NSF, 17 patients). A statistically significant a dose-response association was observed in the cases of SS, GVHD and NSF. Likelihood of clinical improvement from UVA1 phototherapy was very likely for medium- and high-dose regimens in SS, while this level of improvement was only observed in GVHD and NSF patients receiving high-dose UVA1. CONCLUSION: UVA1 phototherapy is effective and safe in the treatment of GVHD, NSF, SS, and mast cell disorders. High-dose regimens appear to be more effective than medium- and low-dose regimens for NSF and GVHD, while medium- and high-dose regimens outperform low-dose UVA1 in SS.

UV-A1 therapy for nephrogenic systemic fibrosis.

BACKGROUND: Nephrogenic systemic fibrosis (NSF) is a rare sclerosing skin condition associated with end-stage renal disease and gadolinium exposure. Therapy for NSF is challenging, with few options other than preventing exposure to gadolinium and improving renal function through transplant. However, in some cases neither of these options is tenable. We report the successful use of UV-A1 phototherapy in 4 patients with NSF. OBSERVATIONS: Four patients with NSF were treated with UV-A1 phototherapy at a tertiary medical center from 2005 through 2007. To our knowledge, it is unique to this series that all patients were receiving hemodialysis before, during, and after therapy with UV-A1. All experienced improvement in the degree of induration, and 2 experienced improvement in mobility of the hands and legs. Total treatments ranged from 22 treatments (with a cumulative dose of 1855 J/cm(2)) to 50 treatments (total UV-A1 exposure, 3850 J/cm(2)). No adverse events were observed. CONCLUSIONS: Although no patient had complete resolution of indurated plaques, the improvement was substantial. For 2 patients, it resulted in a resumption of hand and leg mobility. As a result, UV-A1 therapy may represent a treatment for NSF when kidney transplantation is not an option or is delayed. Limitations of this study include the lack of a controlled trial, lack of quantification of gadolinium levels within tissue, and the lack of a defined grading scale for NSF severity.