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

Journal of Medical Case Reports
Hiba Hasan KhaddourAhmad Hasan

Abstract

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. 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. 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 o...Continue Reading

References

Jan 1, 1990·Journal of the American Academy of Dermatology·R Tan-Lim, J C Bystryn
May 1, 1997·Therapeutic Apheresis : Official Journal of the International Society for Apheresis and the Japanese Society for Apheresis·H YamadaH Ogawa
Dec 2, 2000·Journal of the American Academy of Dermatology·M S TurnerD N Sauder
Mar 7, 2003·Transfusion and Apheresis Science : Official Journal of the World Apheresis Association : Official Journal of the European Society for Haemapheresis·Gustavo MazziBianca Maria Orazi
May 19, 2010·Journal of the European Academy of Dermatology and Venereology : JEADV·L SagiA Barzilai
Feb 3, 2019·BMJ Case Reports·Navin Kumar Devaraj

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biopsy

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