Case report of therapy resistant bullous pemphigoid

Abstract

The authors present a case of a 45- year- old female patient, who has been previously treated for psoriasis with guselkumab. In 2021 after COVID vaccination seropapules on erythematous background and annular blisters developed. Early clinical presentation indicated Sweet syndrome or IgA-mediated autoimmune bullous diseases, however histological examination showed intraepidermal eosinophilic spongiosis with IgG and C3 deposits along the basal membrane – concluding the diagnosis of bullous pemphigoid. ELISA test revealed both anti-BP180 and anti-type VII collagen antibody positivity, the latter being atypical in bullous pemphigoid. Despite the usual immunosuppressant therapy – corticosteroid and later azathioprine – the clinical symptoms showed progression. Due to the therapy resistance, rituximab was administered, which resulted in complete regression of skin sypmtoms. During regular follow-up stable remission has been achieved. As described in the published medical case reports of atypical bullous pemphigoid, we suspect that the main pathogenetic factor could be epitope spreading caused by the 10-year history of psoriasis.