Interest of therapeutic plasmapheresis in a chronic hemodialysis patient with severe bullous pemphigoid.

Bullous pemphigoid is the most common autoimmune blistering disease induced by autoantibodies against basement membrane anchoring proteins (anti-BP-180 and anti-BP-230). The disease generally appears after the age of 70 and is associated with a 23.5% 1-year mortality, especially in diabetics, or in the presence of ischemic heart disease and high anti-BP-180. Treatment starts with topical steroids but some patients may require oral steroids and systemic immunosuppression. We, hereby, discuss a diabetic patient on chronic hemodialysis, with severely relapsed bullous pemphigoid under biotherapy with omalizumab, who was successfully treated with five sessions of double filtration plasmapheresis, thus avoiding the need for systemic steroids.

Ahmadpoor P ，Beck M ，Michel M ，Pambrun E ，Stoebner P ，Moranne O ... -  《-》

Plasmapheresis for the treatment of pemphigus vulgaris and bullous pemphigoid.

Yamada H ，Yaguchi H ，Takamori K ，Ogawa H ... -  《-》

Double filtration plasmapheresis for the treatment of bullous pemphigoid: a three case report.

Kitabata Y ，Sakurane M ，Orita H ，Kamimura M ，Siizaki K ，Narukawa N ，Kaketaka A ，Abe T ，Kobata H ，Akizawa T ... -  《-》

Selective plasma exchange can reduce auto-antibodies in patients with bullous pemphigoid without affecting factor XIII and fibrinogen.

Bullous pemphigoid (BP) is an autoimmune blistering skin disorder characterized by circulating serum IgG antibodies against two hemidesmosomal proteins: BP180 and BP230. Fundamentally, immunosuppressive therapies are administered to treat this disease, but plasmapheresis can be added for refractory patients. We experienced the case of a 63-year-old patient with refractory BP for which we administered double filtration plasmapheresis (DFPP). His skin lesions improved along with decreased IgG BP180 antibodies, but factor XIII (FXIII) and fibrinogen were also reduced by DFPP repetition. Reportedly, deficiency of those factors can cause lethal bleeding. Especially, decreased FXIII cannot be detected by prolongation of bleeding or coagulation time. To prevent further reduction of those factors and bleeding complications, DFPP was switched to selective plasma exchange (SePE), a new modality of plasmapheresis that uses a membrane plasma separator with smaller than ordinary pores. SePE further reduced pathogenic IgG BP180 antibodies, but FXIII and fibrinogen recovered. For this case, we measured the mean of reduction ratios in serum IgG and FXIII both before and after plasmapheresis sessions and detected the decreased levels of FXIII and fibrinogen during DFPP. We were able to switch to SePE from DFPP appropriately before any bleeding event occurred. The utility of SePE was demonstrated, especially for the reduction of pathogenic antibodies with retention of FXIII and fibrinogen, which have the longest half-lives among coagulation factors and which take a long time to recover.

Nasu K ，Hanafusa N ，Nangaku M 《-》

Autoimmune Subepidermal Bullous Diseases of the Skin and Mucosae: Clinical Features, Diagnosis, and Management.

Amber KT ，Murrell DF ，Schmidt E ，Joly P ，Borradori L ... -  《-》