Aggressive refractory pemphigus vulgaris that responded to plasmapheresis: a case report

BACKGROUND: Pemphigus vulgaris is an autoimmune disorder that involves intraepithelial blistering and sores of the skin and mucous membranes. The average age of onset is between 50 and 70 years. Pemphigus rarely occurs in children. It correlates with the level of circulating autoantibodies; therapeutic plasma exchange is hypothesized to remove pathogenic autoantibodies, and this is necessary in refractory severe cases. CASE PRESENTATION: A 14-year-old Asian girl came to our hospital with blisters and erosions all over her body and in the oral mucosa. She was diagnosed with pemphigus vulgaris by skin biopsy about 3 months before hospitalization. She was admitted to the intensive care unit due to aggressively worsening symptoms, extensive lesions, dehydration, and electrolyte imbalance secondary to excess fluid loss from the skin wounds and sepsis secondary to infection of the exposed wounds. She did not respond to prednisone and azathioprine therapy but was successfully treated with plasmapheresis. CONCLUSION: The purpose of this case report is to describe an aggressive presentation of pemphigus vulgaris, especially because the onset of the disease in our patient was at an early age. The disease rarely begins in childhood, and this case report highlights the importance of plasmapheresis as a useful intervention in patients with pemphigus vulgaris who are not responding to conventional therapy, taking into account that there is a paucity of studies showing the effectiveness of plasmapheresis in inducing partial or complete remission in young patients.