Sutton Disease II

Sutton Disease II

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Sutton Disease II is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Aphthous Stomatitis, Recurrent
  • Aphthous Ulcer, Recurrent
  • Major Aphthous Ulcer
  • Major Canker Sore
  • Major Ulcerative Stomatitis
  • Periadenitis Mucosa Necrotica, Recurrent Type II
  • RAU
  • Recurrent Scarring Aphthae
  • von Mikulicz's Aphthae
  • Von Zahorsky's Disease

Disorder Subdivisions

  • None

General Discussion

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.

Symptoms

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).

Causes

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.

Affected Populations

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).

Standard Therapies

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.

Investigational Therapies

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

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com



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.

References

TEXTBOOKS

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.



REVIEW ARTICLES

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.



JOURNAL ARTICLES

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.

Resources

NIH/National Institute of Dental and Craniofacial Research

Building 31, Room 2C39

31 Center Drive, MSC 2290

Bethesda, MD 20892

USA

Tel: (301)496-4261

Fax: (301)480-4098

Tel: (866)232-4528

Email: nidcrinfo@mail.nih.gov

Internet: http://www.nidcr.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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