Aggressive Refractory Pemphigus Vulgaris That Responded to Plasmapheresis

A Case Report

Hiba Hasan Khaddour; Diana Zaher; Triak Kassem; Ahmad Hasan


J Med Case Reports. 2020;14(109) 

In This Article

Discussion and Conclusion


A 14-year-old- girl came to our hospital with blisters and erosions all over her body and in the oral mucosa. She was diagnosed with PV 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 importance of this case comes from the fact that the average age of onset of PV is between 50 and 70 years, and PV rarely occurs in children. Most studies published in the medical literature have reported patients with PV in the age range of 40–80 years. The unusual occurrence of PV in a girl can be explained by the psychological severity of the dire conditions that the patient has experienced in the area where she lives.

PV is a serious and potentially life-threatening disease. It can be fatal because there is a loss of the epidermal barrier and a loss of body fluids, and there are chances of a secondary infection. Early and accurate diagnosis is a prerequisite for immediate therapy to prevent fatal outcomes. The clinical diagnosis must be confirmed by histopathological evaluation.[5]

The use of systemic corticosteroids with or without immunosuppression is the mainstay of treatment of PV. However, there are still some patients who undergo unacceptably high doses of corticosteroids. We cannot taper their high dosages of medications or treat those who with extreme degree of disease.[6]

The role of topical corticosteroids is controversial in PV.[7] The presence of circulating antibodies has an essential role in the pathogenesis of PV. Plasmapheresis is a treatment for reducing the titers of circulating autoantibodies, which has correlated with clinical improvement.[8] Combination therapy of plasmapheresis and immunosuppressants appears to lead to complete remission and brings the disease to a level that can be controlled by corticosteroids. In our patient's case, clinical evaluation was used to monitor the efficacy of treatment, and no adverse effects of plasma exchange have occurred.

Most studies published in the medical literature involving patients with PV and pemphigoid pemphigus have ages between 40 and 80 years. These studies have reported the effectiveness of plasmapheresis by inducing clinical improvement and the possibility of reducing the dose of corticosteroids by a large percentage and showed that the side effects of plasmapheresis are few.[9–12]

The purpose of this case report is to describe an aggressive presentation of PV, especially since the onset of the disease was at an early age and the disease rarely begins in childhood. This case highlights the importance of plasmapheresis as a useful intervention in patients with PV who are not responding to conventional therapy, taking into account that there is a paucity of studies that demonstrated the effectiveness of plasmapheresis in inducing partial or complete remission in young patients.

Patient Perspective

The patient is grateful because she is still alive. She hopes that the dose of corticosteroids will be reduced to the lowest possible dose and that the treatment will not be lifelong. She hopes that the disease will not affect her life course, especially because she is still a child, and that doctors will discover a radical treatment for her illness.