Successful treatment of bullous pemphigoid with pulsed intravenous cyclophosphamide

Abstract

Pulsed therapy with intravenous cyclophosphamide was successful in a patient with bullous pemphigoid whose disease had proved resistant to other treatments.

British Association of Dermatologists’ guidelines for the management of bullous pemphigoid 2012

“…Three cases have been reported in which cyclophosphamide appeared to have a beneficial effect in otherwise refractory BP 82–84 …”

Treatment of subepidermal immunobullous diseases

“…Although cyclophosphamide is often used to treat ocular cicatricial pemphigoid, several case reports have suggested that cyclophosphamide may also be useful in cases of severe or refractory BP when given in either oral daily (1–5 mg/kg/d) or pulse intravenous dosing. 40,41 No large or randomized trials have been performed using this medication.…”

Fatal bullous pemphigoid unresponsive to any therapeutic regimens

“…Our case is unique in terms of the unresponsiveness of BP to conventional therapeutic regimens 1–5 . Even combination of cyclophosamide pulse 6 and immunoadsorption, 7 which are reported to be effective for therapy refractory cases failed to suppress blister formation. Only CysA, a non‐standard therapy in the management of BP, showed some positive short‐term effect.…”

“…Bullous pemphigoid (BP) is an acquired autoimmune subepidermal blistering disease of the skin that predominantly affects the elderly and usually responds effectively to conventional therapeutic regimens 1–7 . We describe a 53‐year‐old woman who presented uncommon clinical and biological features of BP such as young age, mucosal involvement and lack of efficacy to any standard therapies.…”