|55 Kenosia Avenue
Danbury, CT 06810
Toll Free: 1.800.999.6673
The National Organization for Rare Disorders (NORD) web site, its databases, and the contents thereof are copyrighted by NORD. No part of the NORD web site, databases, or the contents may be copied in any way, including but not limited to the following: electronically downloading, storing in a retrieval system, or redistributing for any commercial purposes without the express written permission of NORD. Permission is hereby granted to print one hard copy of the information on an individual disease for your personal use, provided that such content is in no way modified, and the credit for the source (NORD) and NORD’s copyright notice are included on the printed copy. Any other electronic reproduction or other printed versions is strictly prohibited.
The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.
Copyright 1986, 1994, 2003
Sutton disease II is characterized by the recurring eruption of painful inflamed ulcers in the mouth (stomatitis). There may be multiple ulcers of varying sizes. These ulcers in the mouth are commonly called canker sores. Sutton disease II is also known as recurrent aphthous stomatitis. The exact cause of this disease is not fully understood, although it may be due to an abnormal immune response to the bacteria that are normally in the mouth.
The symptoms of Sutton disease II include red, painful ulcers that may appear on the tongue, the lining of the cheeks (buccal mucosa), floor of the mouth, and back of the throat (soft palate). Ulcers may develop in clusters or appear as single lesions scattered throughout the mouth. As many as 15 sores may be present at once. Individuals with Sutton disease II typically experience recurring episodes of mouth sores, usually with 2 to 3 sores during each attack.
The ulcers associated with Sutton disease II may vary in size. When the mouth ulcers associated with Sutton disease first erupt, they usually appear as red (inflamed) shallow erosions. Sores less than 1 centimeter in size are considered small or "minor" ulcers. These smaller sores are the most common form of the disease and usually last for 10 to 14 days. Smaller lesions do not usually leave scars. In some cases larger or "major" ulcers may develop lasting for weeks or months. Typically, these larger sores leave scars.
In severe cases, other symptoms of Sutton disease II may include a general feeling of weakness (malaise), fever, and swollen lymph nodes around the neck and head (lymphadenopathy).
The exact cause of Sutton disease II is not known. Several studies suggest that it may occur because of an abnormal immune response to the bacteria that are normally present in the mouth. Deficiencies of iron, vitamin B12, and folic acid seem to increase an individual's susceptibility to this disease. Stress and local injury may also be involved. There seems to be no relationship between Sutton disease II and menstruation, pregnancy, and/or menopause, nor is it caused by herpes virus, which is the main cause of common canker sores.
Sutton disease II affects more adult females than males. However, before puberty, males and females are equally affected. This disease occurs most frequently in malnourished children or adults whose immune systems are suppressed (i.e., by chemotherapy) or compromised (i.e., acquired immune deficiency syndrome).
Symptoms of the following disorders can be similar to those of Sutton disease II. Comparisons may be useful for a differential diagnosis:
Pemphigus is a group of rare autoimmune skin disorders characterized by the development of blisters in the outer layers of the skin and mucous membranes (i.e., in the mouth). The location and type of blisters vary according to the type of pemphigus. Soft (flaccid) blisters typically develop on the skin of the neck, scalp, underarms, and/or mucous membranes. When they occur in the mouth, they may resemble the lesions of Sutton disease II.
Herpetic oral ulcers, more commonly known as "canker sores," are caused by the herpes virus and appear similar to the sores caused by Sutton disease II. They are usually red and painful and typically heal within a few weeks. Unlike the sores of Sutton disease, herpetic oral ulcers usually appear on the gums around the teeth and on the roof of the mouth.
Recurrent mouth ulcers may also occur in association with other disorders such as cyclic neutropenia, hand-foot-mouth syndrome, Behcet's syndrome, and iron deficiency disorders. (For more information on these disorders, choose "Neutropenia, Cyclic" and "Behcet's" as your search term in the Rare Disease Database.)
The symptomatic treatment of Sutton disease II involves the application of topical anesthetic (i.e., lidocaine viscous) directly on the affected areas or a thorough mouth rinsing with a special anesthetic solution that helps to reduce irritation and pain. The application of topical steroids may also give symptomatic relief. A dental protective paste (i.e., triamcinolone acetonide or Orabase) may prevent teeth, dental appliances and oral fluids from irritating the ulcers.
In severe cases of Sutton disease II, oral and topical steroids may be administered. A mouthrinse that contains the antibiotic tetracycline may also be prescribed to heal painful mouth sores. However, the use of antibiotics and steroids may promote the development of the fungus candida that can cause thrush or oral candidiasis.
If treatment is started early, symptomatic relief may occur during the first day of treatment and new lesions may be healed. Recurring oral ulcers generally require renewed efforts at treatment. Other treatment is symptomatic and supportive.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.
For information about clinical trials being conducted at the National Institutes of Health (NIH) in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
For information about clinical trials sponsored by private sources, contact:
The treatment of Sutton disease II with zinc sulfate supplementation and the drug azathioprine (an immunosuppressant) is being studied in those affected individuals who have not responded to standard medical treatment. More study is needed to determine the long-term safety and effectiveness of these medications for the treatment of Sutton disease II.
PO Box 8126
Gaithersburg, MD 20898-8126
Phone #: 301-251-4925
800 #: 888-205-2311
Home page: http://rarediseases.info.nih.gov/GARD/
Building 31, Room 2C39
31 Center Drive, MSC 2290
Bethesda, MD 20892 USA
Phone #: 301-496-4261
800 #: 866-232-4528
Home page: http://www.nidcr.nih.gov/
Ramos-Caro FA. Sutton Disease. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:138.
Beers MH, Berkow R., eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:756-57.
Berkow R., ed. The Merck Manual-Home Edition. Whitehouse Station, NJ: Merck Research Laboratories; 1997:456.
Bennett JC, Plum F. Eds. Cecil Textbook of Medicine. 20th ed. W.B. Saunders Co., Philadelphia, PA; 1996:646.
Rogers RS 3rd. Pseudo-Behcet's disease. Dermatol Clin. 2003;21:49-61.
Zunt SL. Recurrent aphthous stomatitis. Dermatol Clin. 2003;21:33-39.
Bruce AJ, Rogers RS 3rd. Acute oral ulcers. Dermatol Clin. 2003;21:1-15.
McCarty MA, Garton RA, Jorizzo JL. Complex aphthosis and Behcet's disease. Dermatol Clin. 2003;21:41-48, vi.
Tilliss TS, McDowell JD. Differential diagnosis: is it herpes or aphthous? J Contemp Dent Pract. 2002;3:1-15.
Casiglia JM. Recurrent aphthous stomatitis: etiology, diagnosis, and treatment. Gen Dent. 2002;50:157-66.
Brozovic S, Vucicevic-Boras V, Mravak-Stepetic M, et al. J Oral Pathol Med. 2002;31:106-8.
Brozovic S, Vucicevic-Boras V, Bukovic D. Serum IgA, IgG, IgM, and salivary IgA in recurrent aphthous ulceration. Coll Antropol. 2001;25:633-37.
Eisen D, Lynch DP. Selecting topical and systemic agents for recurrent aphthous stomatitis. Cutis. 2001;68:201-06.
Sistig S, Cekic-Arambasin A, Rabatic S, et al. Natural immunity in recurrent aphthous ulceration. J Oral Pathol Med. 2001;30:275-80.
Report last updated: 2008/04/15 00:00:00 GMT+0