Acute Management of Refractory Bullous Pemphigoid Using Plasma Exchange and Granulocyte-Monocyte-Apheresis: A Case...

Bullous pemphigoid (BP) is a chronic autoimmune blistering disorder, characterized by subepidermal blister formation and intense pruritus, predominantly affecting older adults. Its pathogenesis is complex and only partially understood, involving both humoral and cellular immune mechanisms. Current treatment strategies rely primarily on topical or systemic corticosteroids, depending on disease extent. However, the long-term use of corticosteroids is often constrained by substantial toxicity, prompting the use of steroid-sparing agents such as methotrexate, rituximab, and other immunomodulatory therapies. Nevertheless, the management of BP remains challenging, particularly in refractory cases, due to frequent relapses and treatment-associated adverse events.

We report the case of a 67-year-old woman with long-standing refractory BP and multiple relapses despite various therapeutic interventions, including dupilumab. Due to persistent disease activity, therapeutic plasma exchange was performed, resulting in marked clinical improvement and allowing subsequent maintenance therapy with intravenous immunoglobulin (IVIg). Six months later, however, she developed a severe flare with progressive blistering that remained refractory to high-dose systemic corticosteroids. Following repeat plasma exchange and subsequent granulocyte-monocyte apheresis (GMA), a marked clinical response was observed, with achievement of sustained clinical remission.

In this case of severe refractory BP, sequential extracorporeal therapies were temporally associated with disease control. Plasma exchange followed by IVIg was associated with an initial clinical response, whereas repeat plasma exchange and subsequent GMA coincided with remission during a later inflammatory relapse. Although a causal relationship cannot be established from a single case, these observations suggest that tailored extracorporeal approaches including GMA may represent a valuable therapeutic option in selected patients with severe, treatment-refractory BP and warrant further investigation.