Short-Time Extracorporeal Photochemotherapy in the Treatment of Drug-Resistant Autoimmune Bullous Diseases

Abstract

Background: Bullous pemphigoid (BP) and pemphigus vulgaris (PV) are potentially severe diseases. In drug-resistant PV and pemphigus foliaceus, long-term adjuvant treatment with extracorporeal photochemotherapy (photopheresis, ECP) has been reported to induce remission. Only limited numbers of patients have been reported so far. No information about the effectiveness in drug-resistant BP is available. Patients and Methods: Seven patients with drug-resistant autoimmune bullous diseases have been referred to the photopheresis center of Jena (3 × PV, 3 × BP, 1 × pemphigus foliaceus). The age ranged from 31 to 85 years. ECP was performed on 2 consecutive days once a month. Oral 8-methoxypsoralen was used as photosensitizer. Previous immunosuppressive treatment with either prednisolone or prednisolone/ azathioprine was continued. Results: Complete remission (absence of skin or mucous membrane lesions) was achieved in the 6 patients with PV and BP after 1–4 cycles. In the patient suffering from pemphigus foliaceus, a partial remission (> 50% improvement) was observed; in all except this patient, the immunosuppressive treatment could be tapered. Long-term remission was achieved. No severe side effects were observed. The treatment was well tolerated. Conclusions: Short-time ECP is an effective and safe adjuvant treatment for patients with drug-resistant autoimmune bullous diseases. It can induce remission and allows dose tapering of the immunosuppressive drugs.

Drug‐resistant bullous pemphigoid and inflammatory bowel disease in a pediatric case successfully treated by plasma exchange and extracorporeal photochemotherapy

“…We included ECP in our protocol in order to obtain long-term immunotolerance. Wollina et al [4] showed how all the drugresistant BP patients in their study achieved a complete response after a limited number of ECP cycles alone. Similarly to these authors, we observed a complete clinical response after a limited number of ECP procedures in both acute and chronic GvHD in children (unpublished personal data); we, therefore, opted to include only four ECP procedures in our protocol.…”

“…Two studies [13,14] showed the benefit of plasmapheresis as steroid saving agent; although the literature regarding the use of plasmapheresis in drug-resistant BP is scant [2,3,[15][16][17], it does show that a general improvement in clinical symptoms and remission allows a subsequent reduction in the required dose of corticosteroids. The use of ECP in BP is poorly investigated: only one study [4] has reported a response in drug-resistant BP using a short-time ECP protocol. Inflammatory bowel diseases (IBD) are considered to be systemic autoimmune diseases since they are often associated with extraintestinal manifestations, complications, and other autoimmune disorders.…”

“…Standard care entails oral steroid treatment; the effectiveness of the addition of plasma exchange (PEX) has not been established [1], even though it has been shown to allow the reduction of corticosteroid doses and to improve symptoms in therapy-resistant cases [2,3]. Short-term extracorporeal photochemotherapy (ECP) can provide effective and safe adjuvant treatment for patients with drug-resistant BP [4]. Inflammatory bowel diseases (IBD) are considered to be systemic autoimmune diseases in which growth and nutrition are key issues to be taken into account during treatment with immunosuppressive drugs [5,6].…”

Pemfigusta Konvansiyonel Olmayan Tedavi Yaklaşımları

“…Tüm vakalarda klinik iyileşme görülmüş ve eşzamanlı kullanılan prednizolon ve diğer immünsüpresif ilaçların dozları düşürülebilmiştir (14).…”

Extracorporeal Photochemotherapy as a Challenging Treatment for Cutaneous T-Cell Lymphoma, Acute and Chronic Graft-versus-Host Disease, Organ Rejection and T-Lymphocyte-Mediated Autoimmune Diseases

“…Other Dermatological Diseases ECP treatment has been proposed in several autoimmune dermatological diseases, including pemphigus bullosus and vulgaris, in drug-resistant patients or as an adjuvant therapy [48][49][50], with satisfactory results in 60-75% of patients. Similar findings regarding ECP treatment have been published in epidermolysis bullosa [51], chronic erosive lichen planus [52] and atopic dermatitis [53].…”