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- Pemphigus vulgaris—most common type of pemphigus
- Pemphigus foliaceus
- Paraneoplastic pemphigus—most serious type, usually occurs with cancer
- Family members with pemphigus
- A history of having autoimmune diseases, such as myasthenia gravis, lupus, or thymoma
- Jewish or Mediterranean descent
Regular use of certain drugs, including:
- Lesion may extend deep into the skin
- Blisters usually start in the mouth or on the scalp
- Lesions progress to the face, neck, upper body, armpits, and groin
- Blisters also may occur in the esophagus, rectum, nose, throat, larynx (voice box), eyes, vulva, or rectum
- Surrounding skin may be red or normal color
- Open sores are painful
- Pressure on the blister may cause it to expand into surrounding tissue
- Outer layer of skin can easily be rubbed off
- Blisters are soft and break easily, releasing fluid
- Large areas of skin may open up, increasing the risk of fluid imbalance and infection
- Blisters usually heal without scarring, but skin color may change to brown
- Lesions are superficial
- Blisters are itchy or produce a burning sensation
- Sores are usually not found in the mouth or on other mucus membranes
- Blisters usually first show up on the face, scalp, chest, or upper back
- Blisters open, causing shallow sores
- Skin is red
- Scales and crusts form
- Sunlight may make symptoms worse
- Sores occur on the mucous membranes, in the mouth, eye, and esophagus
- Blisters often appear on palms of hands and soles of feet
- Lesions are itchy or painful
- Skin biopsies of the lesion and surrounding tissue
- Blood and skin tests
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- Steroids—reduce swelling and calm the immune system
- Immunosuppressive medications—decrease the strength of your immune system
- Antibiotics—treat or prevent infection
- Medicated mouthwash—for pain relief
- IV immunoglobulin—immune system elements from a healthy immune system, often given in combination with rituximab (a biologic antineoplastic agent)
- Topical steroids, including injection into specific lesions
American Autoimmune Related Diseases Association, Inc. http://www.aarda.org
International Pemphigus Foundation http://www.pemphigus.org
Canadian Dermatology Association http://www.dermatology.ca
Health Canada http://www.hc-sc.gc.ca
Beers MH, et al. The Merck Manual of Medical Information—Home Edition. 2nd ed. Simon and Schuster, Inc.; 2003.
Chams-Davatchi C, Esmaili N, et al. Randomized controlled open-label trial of four treatment regimens for pemphigus vulgaris. J Am Acad Dermatol. 2007 Oct;57(4):622-628.
Ferri FF. Ferri's Clinical Advisor: Instant Diagnosis and Treatment. 8th ed., St. Louis: Mosby; 2006.
Goldman L. Cecil Textbook of Medicine. 22nd ed. Philadelphia: Saunders; 2004.
Griffith's 5-Minute Clinical Consult. 2001 ed. Lippincott Williams & Wilkins; 2001.
Martin LK, Werth V, et al. Interventions for pemphigus vulgaris and pemphigus foliaceus. Cochrane Database Syst Rev. 2009 Jan 21;(1):CD006263.
Pemphigus. National Institute of Arthritis and Musculoskeletal and Skin Disease (NIAMS) website. Available at: http://www.niams.nih.gov/Health%5FInfo/Pemphigus/default.asp#10. Accessed December 11, 2012.
Pemphigus foliaceus. EBSCO DynaMed website. Available at: https://dynamed.ebscohost.com/about/about-us. Updated May 20, 2011. Accessed December 11, 2012.
Pemphigus vulgaris. EBSCO DynaMed website. Available at: https://dynamed.ebscohost.com/about/about-us. Updated May 20, 2011. Accessed December 11, 2012.
- Reviewer: Michael Woods, MD
- Review Date: 12/2013
- Update Date: 12/22/2013