Systemic Pemphigus Vulgaris: A Case Report.
- Author:
Bommie SEO
1
;
Je Won SEO
;
Deuk Young OH
;
Sang Tae AHN
;
Jong Won RHIE
Author Information
1. Department of Plastic and Reconstructive Surgery, College of Medicine, The Catholic University of Korea, Seoul, Korea. rhie@catholic.ac.kr
- Publication Type:Case Report
- Keywords:
Pemphigus;
Pemphigus vulgaris
- MeSH:
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
- From:Journal of the Korean Society of Plastic and Reconstructive Surgeons
2011;38(5):687-690
- CountryRepublic of Korea
- Language:English
-
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.
