The Management of Pemphigus Vulgaris in a Burn Intensive Care Unit

Abstract

Pemphigus vulgaris is a rare, potentially fatal, autoimmune blistering disease of the skin and mucous membranes. Treatment of this disease is problematic because of a lack of high-grade, evidence-based recommendations, the side-effect profiles of the therapies available, and the extensive supportive care that afflicted patients require. The authors present the unfortunate course of a patient with severe pemphigus vulgaris who was admitted to the U.S. Army Institute of Surgical Research Burn Center, to demonstrate the potential complications of therapy. Given the patient's complex course, the authors reviewed the literature and share in this article the most up-to-date treatment recommendations for patients with pemphigus vulgaris. The authors' review of the literature supports using conventional therapy consisting of high-dose corticosteroids and an adjuvant immunosuppressant for mild to moderate cases of pemphigus vulgaris. The immunosuppresants recommended are mycophenolate mofetil, azathioprine, and cyclophosphamide, in order of preference, based on their side-effect profiles and steroid-sparing effects. For severe or recalcitrant cases of pemphigus vulgaris, the authors recommend adding rituximab as early as possible. If increased risk of infection is of particular concern, the use of intravenous immunoglobulin in place of rituximab is advised.

Pemphigus Vulgaris

“…Administration of systemic corticosteroids for the treatment of pemphigus vulgaris can lead to a variety of adverse effects that can be serious and sometimes fatal. Reported adverse effects include but are not limited to diabetes, osteoporosis, Cushing syndrome, and increased susceptibility to infection 11 . The main causes of fatality are sepsis and lung embolism, one example being described in a case report by Sakallioglu et al 6 In our case, the patient developed severe immunosuppression that led to multiple infections including hospital-acquired pneumonia and bacteremia.…”

“…Nonsteroidal immunomodulatory agents have emerged as a treatment option for patients who are not responding to the standard corticosteroid treatment 13 . This was demonstrated in a case report by Miletta et al 11 in which pemphigus vulgaris was successfully treated with a combination of high-dose corticosteroids and an adjuvant immunosuppressant. In severe cases, high doses of corticosteroids that are necessary to treat the disease can lead to worsening of complications.…”

Oral management of pemphigus vulgaris in the intensive care unit

“…Specialized care and permanent surveillance are associated with a reduction in the mortality of critically ill patients 19 . Treatment of patients with PV is considered complex due to the support required and the risk of secondary infection 20 . In addition, the fact that oral lesions heal slowly, compared to skin lesions, reinforces the need for individualized and specialized dental care 8 …”

Wound Care in Immunobullous Disease

“…Frequently in pemphigus vulgaris and bullous pemphigoid, the body is covered with intact bullae and partial-thickness wounds from head to toe. Serous sanguineous fluid drains onto the linens, which exacerbates pain since once it dries it becomes stuck and unstuck to bed sheets and bandages [54] (Figure 7).…”

“…At the start of the wound care, large abdominal dressings were also applied over the Xerofoam gauze to absorb the drainage. Above that, various kinds of stretch bandages or nets were placed to secure the Wound Care in Immunobullous Disease http://dx.doi.org/10.5772/intechopen.71937 dressing [54]. It is important to keep the skin surface moist and not open to air for healing and pain management.…”