전신성 심상성 천포창의 치험례 = Systemic Pemphigus Vulgaris: A Case Report

다국어 초록 (Multilingual Abstract)

Purpose: Pemphigus vulgaris, a rare autoimmune blistering disease of the skin and mucous membranes remains a challenging disease to treat. Management is focused on immunotherapy against autoimmune antibodies that target keratinocyte cell adhesion molecules,and antibiotics preventing secondary infections. There is no established dressing protocol and skin is usually manipulated the least amount possible in order to minimize irritation. The authors suggest that early initiation of aggressive bathing and debridement of skin lesions, with nutritional support, is essential in accelerating resolution.
Methods: A 40 year-old male previously diagnosed with pemphigus vulgaris was admitted due to exacerbation of mucocutaneous lesions involving the epidermis and mucosa of the whole body. Steroids, immunosuppressants,intravenous immunoglobulin and antibiotics were administrated, but infection and de-epithelialization progressed,while his general condition deteriorated with a weight loss of over 20 kilograms. The plastic surgery department intervened with daily bathing, debridement of unhealthy debris and non-traumatizing coverage of growing epithelium. Total parenteral nutrition and mobilization with rehabilitation therapy was initiated as early as possible.
Results: After bathing, healthy epithelium gradually covered the patient’s entire body, while his general condition improved with a corresponding weight gain of 14kgs.
Conclusion: Treatment of pemphigus vulgaris focuses on immunotherapy and infection control. However, an equal amount of attention should be laid on early intervention with daily dressings including bathing and irrigation,nutritional support, and exercise as this accelerates resolution of existing infections, promotes healthy epithelialization and leads to faster recovery.

참고문헌 (Reference)

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