Granuloma Annulare

Granuloma Annulare

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Granuloma Annulare 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

  • Lichen Annularis

Disorder Subdivisions

  • Localized Granuloma Annulare
  • Generalized (Disseminated) Granuloma Annulare
  • Perforating Granuloma Annulare
  • Linear Granuloma Annulare
  • Subcutaneous Granuloma Annulare

General Discussion

Granuloma annulare is a chronic degenerative skin disorder. The most common form is localized granuloma annulare, which is characterized by the presence of small, firm red or yellow colored bumps (nodules or papules) that appear arranged in a ring on the skin. In most cases, the sizes of the lesions range from one to five centimeters. The most commonly affected sites include the feet, hands, and fingers. In addition to the localized form, there are four less common forms: generalized or disseminated, linear, perforating, and subcutaneous. The lesions associated with granuloma annulare usually disappear without treatment (spontaneous remission). However, the lesions often reappear. The exact cause of granuloma annulare is unknown.

Symptoms

Five recognized forms or clinical variants of granuloma annulare have been identified. The most common form is localized granuloma annulare. The four other forms (i.e., generalized or disseminated, linear, perforating, and subcutaneous) occur less often than the localized form. All forms of granuloma annulare are characterized by small, firm bumps (nodules or papules) arranged in a ring on the skin. These bumps are usually skin-colored or slightly red or yellow. Most cases of granuloma annulare clear up without treatment (spontaneous resolution). However, recurrences are common.



Granuloma annulare may affect any area of the body. The fingers, hands and feet are the areas most often affected. In most cases both sides of the body are affected (symmetrical). Other commonly affected areas include the forehead, neck and abdomen. Localized granuloma annulare normally affects one specific area of the body.



Generalized or disseminated granuloma annulare may affect several areas of the body at one time. The bumps associated with this form of GA are usually smaller and more numerous than those associated with the localized form. These bumps may also be itchy (pruritic).



Subcutaneous granuloma annulare may present as a solitary, painless mass or nodule underneath the skin (subcutaneous). The scalp, arms, and legs are most often affected. Children are affected more often than adults.



Perforating granuloma annulare is characterized by bumps or pustules that develop a yellow center. These lesions may leak a clear fluid, become crusted and eventually leave a scar. These lesions may come together (coalesce) to form larger plaques.



Linear granuloma is an extremely rare form of granuloma annulare that most often affects the fingers.

Causes

The exact cause of granuloma annulare is unknown (idiopathic). Numerous theories exist linking the cause to trauma, sun exposure, thyroid disease, tuberculosis, and various viral infections. However, no definitive proof has been shown for any of these theories.



The disseminated type of the disorder, which affects large areas of the body, may be associated with diabetes mellitus. Granuloma annulare may also be a complication of pseudorheumatoid nodules or shingles (herpes zoster). Some forms of GA tend to run in families (familial), but the exact mode of inheritance has not yet been determined. (For more information on the above disorders, chooses "Diabetes Mellitus" and "Herpes Zoster" as your search terms in the Rare Disease Database.)

Affected Populations

Granuloma annulare occurs more often in females than males. The disorder can affect people of any age, but occurs most frequently in children and young adults. The prevalence of granuloma annulare in the general population is unknown. Localized granuloma annulare occurs more often than the others forms.

Standard Therapies

In many cases, the eruptions of granuloma annulare disappear without treatment (spontaneous remission). Therefore, many affected individuals do not require specific treatment. However, the episodes of the disorder may recur. Treatments for chronic forms of the disorder include corticosteroid drugs such as dapsone and isotretinoin, which is the synthetic form of retinoic acid (related to vitamin A).

Investigational Therapies

A procedure known as psoralen plus ultraviolet A radiation (PUVA) may also be used to treat individuals with granuloma annulare, especially the generalized form. This procedure may be referred to as photochemotherapy. During this procedure, affected individuals topically apply the drug psoralen. Affected individuals are then treated with ultraviolet A light, which activates the drug. PUVA therapy has been effective in treating individuals with granuloma annulare. More research is necessary to determine the long-term safety and effectiveness of topical PUVA therapy for individuals with granuloma annulare.



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 web site.



For information about clinical trials being conducted at the NIH Clinical Center 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

References

TEXTBOOKS

Champion RH, et al., eds. Textbook of Dermatology. 5th ed. Cambridge, MA: Blackwell Scientific Publications; 1992:2027-33.



JOURNAL ARTICLES

Smith KJ, et al. Treatment of disseminated granuloma annulare with 5-lipooxygenase inhibitor and vitamin E. Br J Dermatol. 2002;146:667-70.



Hsu S, et al. Differential diagnosis of annular lesions. Am Fam Physician. 2001;64:289-96.



Houcke-Bruge C, et al. Granuloma annulare following BCG vaccination. Ann Dermatol Venereol. 2001;128:541-4.



Grandmann-Kollmann M, et al. Cream psoralen plus ultraviolet A therapy for granuloma annulare. Br J Dermatol. 2001;144:996-9.



Schulze-Dirks A, Petzoldt D. Granuloma annulare disseminatum: successful therapy with fumaric acid ester. Hautarzt. 2001;52:228-30.



Grogg KL, Nascimento AG. Subcutaneous granuloma annulare in childhood: clinicopathologic features in 34 cases. Pediatrics. 2001;107:E42.



Setterfield J, et al. Generalised granuloma annulare successfully treated with PUVA. Clin Exp Dermatol. 1999;24:458-60.



Chung S, et al. Subcutaneous granuloma annulare: MR imaging features in six children and literature review. Radiology. 1999;210:845-9.



Vandevenne JE, et al. Subcutaneous granuloma annulare: MR imaging and literature review. Eur Radiol. 1998;1363-5.



Felner EI, et al. Subcutaneous granuloma annulare: a review of 47 cases. Pediatrics. 1997;100:965-7.



Barron DF, et al. Granuloma annulare. A clinical review. Lippincotts Prim Care Pract. 1997;1:33-9.



Penas JF, et al. Perforating granuloma annulare. Int J Dermatol. 1997;36:340-8.



Davids JR, et al. Subcutaneous granuloma annulare: recognition and treatment. J Pediatr Orthop. 1993;13:582-6.



Samlaska CP, et al. Generalized perforating granuloma annulare. J Am Acad Dermatol. 1992;27:319-22.



Steiner A, et al. Sulfone treatment of granuloma annulare. J Am Acad Dermatol. 1985;13:1004-8.



Schleicher SM, et al. Resolution of disseminated granuloma annulare following isotretinoin therapy. Cutis. 1985;36:147-8.



Muhlemann MF, et al. Localized granuloma annulare is associated with insulin-dependent diabetes mellitus. Br J Dermatol. 1984;111:325-9.

Resources

NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases

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Genetic and Rare Diseases (GARD) Information Center

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TDD: (888)205-3223

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



For a Complete Report

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