Synonyms

• HS
• Hidradenitis Axillaris

Disorder Subdivisions

• None

Related Disorders List

Information on the following diseases can be found in the Related Disorders section of this report:

• Furunculosis

General Discussion

Hidradenitis suppurativa is a chronic, pus-producing (suppurative), scarring (cicatricial) disease process that occurs due to obstruction of hair follicles and secondary infection and inflammation of certain sweat glands (apocrine glands), particularly those under the arms (axillae) or within the anal/genital (anogenital) region. The disease is characterized by the development of recurrent, boil-like nodular lesions and deep pus-containing pockets of infection (abscesses) that may eventually rupture through the skin. Healing of affected areas is typically associated with progressive scarring (fibrosis). The specific underlying cause of hidradenitis suppurativa is unknown.

Symptoms

Hidradenitis suppurativa is a chronic, inflammatory disease affecting sweat glands known as apocrine glands. These sweat glands, located primarily under the arms (axillae) and within the anal and genital (anogenital) region, typically begin to function during puberty. In most individuals with hidradenitis suppurativa, recurrent lesions develop in the axillae, groin, vulva, or anal region. However, in some cases, the condition may involve sweat glands of the breasts, scalp, or other areas. Evidence suggests that females are more commonly affected in the axillary or vulvar region, while males primarily have involvement around the anal region.



Although the age at onset may vary, symptoms often become apparent during puberty or early adulthood. The disease may tend to have a duration of many years, with periodic improvement and worsening of symptoms.



In some cases, early symptoms, such as itching or discomfort, may precede the condition's characteristic manifestations. In those with hidradenitis suppurativa, obstruction (occlusion) of hair follicles and secondary bacterial infection of apocrine glands lead to the development of tender or painful, reddish nodules and pus-containing cavities of infection (abscesses). Abscesses may spontaneously rupture through the skin, draining pus that may be mingled with a clear fluid (seropurulent). With healing, scar tissue forms and new lesions may develop adjacent to the initial nodules. In some severe cases, deep abscesses may drain via abnormal channels deep within skin tissues that connect them to the body's surface (sinus tract formation). Repeated healing and recurrences result in progressive scarring of affected tissues that may appear as thick, cordlike bands. In extremely severe cases, the condition may become extensive and potentially disabling.

Causes

The exact underlying cause of hidradenitis suppurativa is unknown. Symptoms result from abnormal obstruction (occlusion) of hair follicles and secondary bacterial infection and inflammation of apocrine glands, with subsequent rupture of the ducts, spread of infection, and scarring.



Some affected individuals have a positive family history of the disease, suggesting that genetic factors may play some causative role in some cases. In addition, some researchers suggest that certain environmental factors may be a factor in triggering disease onset in some cases, such as cigarette smoking or the use of certain skin irritants (e.g., particular hair removers or deodorants).

Affected Populations

Hidradenitis suppurativa affects females more commonly than males. The condition typically becomes apparent during puberty or later.

Related Disorders

Symptoms of the following disorders may be similar to those of hidradenitis suppurativa. Comparisons may be useful for a differential diagnosis:



Furunculosis is characterized by the development of inflammatory nodules known as boils or furuncles due to infection with staphylococcus bacteria. Boils are red, tender or painful lumps that discharge a central core of tissue and pus. They may sometimes recur. Contributing factors that make a person vulnerable to boils may include obesity, diabetes mellitis, and poor hygiene.



There are a number of additional diseases that may be characterized by symptoms similar to those associated with hidradenitis suppurativa. (For further information, choose the specific disease name in question as your search term in the Rare Disease Database.)

Standard Therapies

Individuals with hidradenitis suppurativa should avoid using antiperspirants or other skin irritants or wearing tight-fitting clothing. Depending upon disease severity and other factors, physicians may recommend various treatments or treatment combinations. Such measures may include the application of warm compresses, incision and drainage of individual abscesses, and the use of certain topical or systemic antibiotic medications. In some cases, corticosteroid injections may be recommended for isolated lesions. In addition, surgical measures (e.g., surgical removal [excision] and skin grafting of affected areas) may be necessary in cases of extensive, persistent disease. Additional therapies may also be recommended in some cases.

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 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

McKusick VA, ed. Online Mendelian Inheritance in Man (OMIM). Baltimore, MD: The Johns Hopkins University; Entry No: 142690, Last Update 3/13/01.



TEXTBOOKS

Behrman RE, et al., eds. Nelson Textbook of Pediatrics. 16th ed. Philadelphia, Pa; W.B. Saunders Company; 2000:2020-21.



Beers MH, et al., eds. The Merck Manual. 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:799-800.



Fauci AS, et al., eds. Harrison's Principles of Internal Medicine. 14th ed. New York, NY: McGraw-Hill Companies, Inc.; 1998:828.



JOURNAL ARTICLES

Elwood ET, et al. Negative-pressure dressings in the treatment of hidradenitis suppurativa. Ann Plast Surg. 2001;46:49-51.



Bohn J, et al. Surgical treatment of hidradenitis suppurativa. Scand J Plast Reconstr Surg Hand Surg. 2001;35:305-09.



Mengesha YM, et al. Prepubertal hidradenitis suppurativa: two case reports and review of the literature. Pediatr Dermatol. 1999;16:292-96.



Konig A, et al. Cigarette smoking as a triggering factor of hidradenitis suppurativa. Dermatology. 1999;198:261-64.



Boer J, et al. Long-term results of isotretinoin in the treatment of 68 patients with hidradenitis suppurativa. J Am Acad Dermatol. 1999;40:73-76.



Brown TJ, et al. Hidradenitis suppurativa. South Med J. 1998;91:1107-14.



Brown CF, et al. Hidradenitis suppurativa of the anogenital region: response to isotretinoin. Am J Obstet Gynecol. 1988;158:12-15.



Mortimer PS, et al. A double-blind controlled cross-over trial of cyproterone acetate in females with hidradenitis suppurativa. Br J Dermatol. 1986;115:263-68.



Sawers RS, et al. Control of hidradenitis suppurativa in women using combined antiandrogen (cyproterone acetate) and oestrogen therapy. Br J Dermatol. 1986; 115:269-74.



Watson JD. Hidradenitis suppurativa--a clinical review. Br J Plast Surg. 1985;38:567-69.



Dicken CH, et al. Evaluation of isotretinoin treatment of hidradenitis suppurativa. J Am Acad Dermatol. 1984;11:500-02.

Resources

NIH/NationaI Institute of Arthritis and Musculoskeletal and Skin Diseases

Information Clearinghouse

One AMS Circle

Bethesda, MD 20892-3675

USA

Tel: (301)495-4484

Fax: (301)718-6366

Tel: (877)226-4267

TDD: (301)565-2966

Email: NIAMSinfo@mail.nih.gov

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



NIH/National Institute of Allergy and Infectious Diseases

Office of Communications and Government Relations

6610 Rockledge Drive, MSC 6612

Bethesda, MD 20892-6612

Tel: (301)496-5717

Fax: (301)402-3573

Tel: (866)284-4107

TDD: (800)877-8339

Email: ocpostoffice@niaid.nih.gov

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



HS-USA, Inc.

7362 High Hill Dr

Brighton, MI 48116-9143

USA

Tel: (810)231-3419

Email: info@hs-usa.org

Internet: http://www.hs-usa.org



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

Email: http://rarediseases.info.nih.gov/GARD/EmailForm.aspx

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



Hidradenitis Suppurativa Foundation, Inc

P.O. Box 861

Valley Center, CA 92082

Tel: (858)901-4747

Fax: (619)239-3271

Email: info@hs-foundation.org

Internet: http://www.hs-foundation.org