[Treatment of connective tissue disorder-related acral syndromes using UVA-1 phototherapy. An open study of 11 cases].
BACKGROUND
UVA-1 phototherapy is used to treat various connective tissues disorders including systemic lupus erythematosus (SLE) and systemic sclerosis (SS). We conducted an open study to investigate the efficacy of this therapy on connective tissue disorder-related acrosyndrome.


METHODS
Eleven patients with Raynaud's phenomenon refractory to the standard therapy (six SLE and five SS) were treated with UVA-1 in an open study. Whole-body phototherapy was given in seven cases but was restricted to the hands in four cases. The results were evaluated by comparing status before and after treatment using semi-quantitative tools in terms of daily frequency and intensity of spastic phenomena, pain, overall patient satisfaction, improvement of cutaneous lesions, trophic disorders and distal cutaneous flexibility for patients with ScS.


RESULTS
After treatment, Raynaud's phenomenon improved in terms of both frequency and severity in 9/11 patients (82%: 4/6 SS and 5/5 SLE). Likewise, pain decreased in 8/11 cases (73%: 3/6 SS and 5/5 SLE). 7/12 patients felt their condition had improved (64%: 3/6 SS and 4/5 SLE). Cutaneous lesions improved in 5/11 patients (45%: 2/6 SS and 3/5 SLE), especially for lupus-related lesions including chilblains and in idiopathic chilblains that totally subsided within one month. Digital ulcers improved in all cases, with complete healing in 3/4 patients (75%). In SS, cutaneous flexibility significantly improved in 2/6 cases (33%). No major adverse effects were seen in patients treated with hand-only phototherapy but a slight and spontaneously reversible cutaneous rash, reminiscent of lupus lesions, occurred in one female patient receiving whole-body phototherapy.


CONCLUSIONS
This study is the first to provide a precise evaluation of the efficiency of UVA-1 phototherapy on connective tissue disorder-related acrosyndrome. This therapeutic effect is not necessarily restricted to the laboratory effects of UVA-1 since the favourable impact of infrared radiation and a placebo effect cannot be ruled out. Although its methodological limitations are obvious, our study also confirms dare data in the recent literature data by demonstrating significant improvement in cutaneous lesions, trophic ulcers and Raynaud's phenomenon in patients presenting connective tissue disorders, including SS, without any major adverse effects.


CONCLUSIONS
Although these preliminary results remain to be confirmed by large-scale, randomized studies, UVA-1 phototherapy clearly offers a new and valuable therapeutic option in connective tissue disorders associated with acral manifestations and/or lesions, including SLE and SS.
Service de dermatologie, université Montpellier 1, hôpital Saint-Eloi, 80, avenue Augustin-Fliche, 34295 Montpellier cedex 5, France.
C
C
Comte
D
D
Bessis
E
E
Picot
J-L
JL
Peyron
B
B
Guillot
O
O
Dereure
D003240:Connective Tissue Diseases / Q000532:radiotherapy*; D005385:Fingers; D006225:Hand; D006801:Humans; D010789:Phototherapy / Q000379:methods*; D011879:Radiotherapy Dosage; D011928:Raynaud Disease / Q000532:radiotherapy*; D045743:Scleroderma, Diffuse / Q000473:pathology / Q000532:radiotherapy; D014034:Toes; D016896:Treatment Outcome; D014467:Ultraviolet Therapy / Q000379:methods*