Persistent Urticaria Heralding the Onset of Bullous Pemphigoid

Bullous pemphigoid is a common autoimmune blistering disorder that predominantly affects older adults aged 65 and older. While most cases present with a tense blistering eruption, characteristic of the disease, there is a lesser-known pre-bullous phase of the condition that can present insidiously with urticaria and intense pruritus, which can last for several weeks or months. Failure to consider this entity in patients with treatment-refractory, persistent urticaria or itch may lead to delayed diagnosis and poor quality of life. We describe a case of a 79-year-old gentleman who presented with a 3-week history of generalized urticaria on a background of intense pruritus for 4 months. His diagnosis was eventually clinched when he developed blistering lesions on an urticated base, with serum indirect immunofluorescence demonstrating anti-basement membrane zone IgG antibodies. He was commenced on immunomodulatory treatment with moderate doses of oral corticosteroids and doxycycline, with good clinical recovery thereafter. This case vignette highlights several learning points pertaining to the recognition and management of patients with urticarial manifestations in the pre-bullous phase of this common autoimmune blistering disease.