P074 Pemphigus vulgaris management after COVID-19: challenges of low- and middle-income countries

Abstract Pemphigus vulgaris is a rare autoimmune skin blistering disorder. Corticosteroids have remained the cornerstone of management plans for decades. Recently rituximab has established its central place as steroid-sparing agent and has revolutionized treatment. However, it is beyond the reach of many patients in low- and middle-income countries, and there are drastic implications when a patient with pemphigus vulgaris being managed with high-dose corticosteroid in combination with a steroid-sparing immunosuppressant contracts COVID-19 infection. A 55-year-old man, with no known comorbidities, presented with a 1-year history of flaccid blisters and erosions in the mouth, head and neck region. He was initially seen by a local general practitioner and was given topical steroids and short courses of prednisolone. These provided some relief but his disease never remitted. He was not compliant with medication and noticed aggravation of the condition for the last 1 month. He had severe clinical features suggestive of pemphigus vulgaris when he presented to the accident and emergency department of a tertiary care hospital. His Pemphigus Disease Area Index score was calculated to be 104. He was admitted and started on high-dose systemic corticosteroids and azathioprine along with aseptic dressings on open wounds. Simultaneously he had skin biopsies for histopathology of an intact fresh blister and perilesional skin for direct immunofluorescence, which were consistent with pemphigus vulgaris. The patient was initially planned to receive rituximab as first-line treatment to induce remission, but he deferred due to financial constraints. Funds were generated to procure the drug. However, during investigation his QuantiFERON TB Gold test was positive, which was considered a contraindication for rituximab. He was managed with meticulous twice-daily change of dressings along with high-dose immunosuppressants. This led to disease control, with no new lesion eruptions. The patient was discharged with regular follow-up advice. Unfortunately, he contracted severe COVID-19 infection when prednisolone tapering had just started, and died due to its complications. We would like to highlight the significant challenges faced in the management of this case, including inadequate awareness among primary care physicians, high endemic rate of tuberculosis in low- and middle-income countries, and resorting to high-dose steroids and other immunosuppressants for disease control. There is lack of research on low-dose rituximab, which might be considered as a viable option in patients with chronic infections, while balancing risks and benefits for saving a patient’s life. Furthermore, research is needed to understand the long-term implications of COVID-19 on pemphigus vulgaris.