J Korean Soc Plast Reconstr Surg.
2011 Sep;38(5):687-690.
Systemic Pemphigus Vulgaris: A Case Report
- Affiliations
-
- 1Department of Plastic and Reconstructive Surgery, College of Medicine, The Catholic University of Korea, Seoul, Korea. rhie@catholic.ac.kr
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 14 kgs.
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.
Keyword
MeSH Terms
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Anti-Bacterial Agents
Antibodies
Bandages
Baths
Blister
Cell Adhesion Molecules
Coinfection
Debridement
Early Intervention (Education)
Epidermis
Epithelium
Humans
Immunoglobulins
Immunosuppressive Agents
Immunotherapy
Infection Control
Keratinocytes
Male
Mucous Membrane
Nutritional Support
Parenteral Nutrition, Total
Pemphigus
Skin
Steroids
Surgery, Plastic
Weight Gain
Weight Loss
Anti-Bacterial Agents
Antibodies
Cell Adhesion Molecules
Immunoglobulins
Immunosuppressive Agents
Steroids
