P01: ‘Age is just a number’: a rare facial blistering eruption in an 18-year-old female

Emma Porter,¹ Irene Timoney,¹ Niamh Leonard,² Aleem Ud-din¹ and Maeve Lynch¹

¹University Hospital Limerick, Limerick, Ireland; and ²St James’ Hospital, Dublin, Ireland

An 18-year-old female presented reporting a 1-month history of intensely itchy blisters affecting the head and neck. She had a history of mild eczema and was otherwise well. On examination, she had extensive erosions localized to the face, neck and frontal scalp with a few intact bullae. The mucosa was normal. Over 2 weeks bullae emerged on the abdomen and back. Initial biopsy showed ulceration and dermal necrosis. Subsequent biopsy demonstrated subepidermal blisters with polymorphs and eosinophils. Direct immunofluorescence demonstrated strong linear IgG and C3 along the dermoepidermal junction on three separate occasions, confirming a diagnosis of pemphigoid. Pemphigoid antibodies were present (1 : 10) on indirect immunofluorescence. Treatment with prednisolone, dapsone up to 150 mg daily, and doxycycline was commenced shortly after presentation, with clinical improvement allowing for tapering of corticosteroids over a period of 11 months to date. Considered predominantly a disease of elderly people, pemphigoid is an autoimmune blistering condition rarely seen in young people (Persson M, Harman KE, Vinogradova Y et al. Incidence, prevalence and mortality of bullous pemphigoid in England 1998–2017: a population-based cohort study. Br J Dermatol 2021; 184: 68–77). Late adolescent presentation is scarcely reported (Patsatsi A, Kyriakou A, Werth VP. Bullous pemphigoid in adolescence. Pediatr Dermatol 2019; 36: 184–8). Clinical presentation and response to treatment may differ from more classic pemphigoid seen in older age. The treatment of choice is firstly systemic corticosteroids, with early addition of steroid-sparing immunomodulators. Prognosis in young adults and children is good (Persson et al.). This case illustrates a rare and visually striking presentation of pemphigoid in a young adult, an important differential to consider, even though it is unusual in this age group.