Ecthyma-gangrenosum-like rare atypical presentation of bullous pemphigoid in a young patient with neurological disorder treated with rituximab

Abstract

Bullous pemphigoid (BP) is an autoimmune blistering disorder characterized by pruritic tense bullae, predominantly affecting the elderly. Several clinical variants have been described. Here, we report a rare ecthyma-gangrenosum-like atypical presentation of bullous pemphigoid in a young epileptic patient. A 33-year-old male farmer presented with multiple pruritic and painful erosions covered with thick adherent necrotic crust over the feet, legs, and upper limbs, sparing other body areas and mucosae, for 15 days. He reported a history of multiple tense blisters over the trunk three months earlier that had resolved with systemic corticosteroids. The patient had been receiving antiepileptic medications for eight years for generalized tonic-clonic seizures. Diagnosis of bullous pemphigoid was confirmed by characteristic complement C3c deposits at the basement membrane zone on direct immunofluorescence (DIF) of perilesional skin and subepidermal cleavage on histopathology from the earlier episode. There was no significant improvement with systemic corticosteroids, antibiotics, and supportive therapy. He was subsequently treated with rituximab, resulting in marked improvement within one month. This case of ecthyma-gangrenosum-like bullous pemphigoid is reported as it was a hitherto rarely described morphological variant occurring in a young patient with neurological association and for its excellent response to rituximab when conventional treatment was unsatisfactory.