Extracorporeal photochemotherapy in systemic sclerosis and severe morphea

Extracorporeal photochemotherapy (EP) is a new immune-modulating therapy which combines cytapheresis and extracorporeal irradiation of leukocytes by UVA. Recently, patients with systemic sclerosis (SS) have been treated by this method, but the efficacy of EP is still controversial.

Our purpose was to evaluate the efficacy and safety of EP in SS and in severe morphea.

In an open study, 7 patients with SS and 2 patients with severe morphea were treated by EP and were evaluated after 6 months of treatment. No other treatments were allowed during this period.

Except one vasovagal reaction, EP was well tolerated in all patients. The surface of the cutaneous sclerosis was unchanged in 3 of the 7 patients with SS, whereas it was aggravated in the remaining 4 patients. There was no improvement of the visceral involvement of SS in these patients. Although the duration of SS was less than 4 years in 6 out of 7 patients, we did not observe any significant benefit from EP in SS patients. One patient with generalized morphea had stopped EP after 3 months because of loss of vascular access and could not be evaluated. In the last patient with linear morphea of the upper limbs, a reduction in the number of morphea plaques was observed, and the remaining lesions were less visible after 16 months of EP.

The present results do not corroborate those previously published with regard to the efficiency of EP in SS of recent onset. The efficacy of ECP in morphea needs further confirmation.