Hidradenitis Suppurativa

Hidradenitis Suppurativa

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Hidradenitis Suppurativa 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

  • HS
  • hidradenitis axillaris
  • acne inversa
  • apocrine acne
  • acne conglobata
  • apocrinitis
  • Verneuil’s disease
  • Velpeau’s disease
  • Fox-den disease
  • pyodermia sinifica fistulans

Disorder Subdivisions

  • Hurley stage I
  • Hurley stage II
  • Hurley stage III

General Discussion

Summary

Hidradenitis suppurativa (HS) is a chronic condition characterized by swollen, painful lesions, occurring in the armpit (axillae), groin, anal, and breast regions. This disease occurs due to obstruction of hair follicles and secondary infection and sometimes inflammation of certain sweat glands (apocrine glands). It is a progressive disease where single boil-like, pus-filled abscesses become hard lumps, then painful, deep-seated, often inflamed clusters of lesions with chronic seepage. Healing of affected areas is typically associated with progressive scarring (fibrosis). Formation of chronic epithelialized, sometimes interconnected, sinus tracts occur in severe disease. This often leads to the entrapment of perspiration and bacteria in the surrounding tissue, which causes the inflammation and infection. HS can be extremely painful and debilitating but is rarely life threatening; only occurring when the bacteria infection leads to an overwhelming systemic infection in an individual with a weakened immune system.



Introduction

HS was once thought to be a rare disorder because only the most severe cases were reported. HS was first described in the medical literature by Velpeau in 1839 and was considered an extremely severe form of acne (acne inversa). It most often presents at puberty because changes in hair follicles triggered by the surge in sex hormones. The condition may also begin to occur in patients of any age after puberty, but rarely before. Cases vary in severity, but all require some treatment and management. For mild cases, home remedies may be all that is needed. For severe forms however, daily medication is often prescribed and radical surgery may eventually be recommended. Early diagnosis and treatment of HS is important because it can help manage symptoms and prevent new boils and lesions from forming.

Symptoms

HS is a chronic, inflammatory disease affecting sweat glands known as apocrine glands. In most individuals with HS, 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 has a duration of many years, with periodic improvement and worsening of symptoms. Many things can trigger flare-ups of this disease including menstruation for women, weight gain, stress, hormonal changes, heat, and perspiration. In some cases, early symptoms, such as itching or discomfort, may precede the condition's characteristic manifestations.



There are 3 clinical stages of HS. Each stage is best treated with different therapies. Ideally, treatment would stop the progression to stage 3 for as long as possible. However, HS has no cure and the disease will eventually follow its course. These stages are as follows:



Harley stage 1: In some cases, early symptoms, such as itching or discomfort, may precede the condition's characteristic manifestations.



Harley stage 2: Recurrent abscesses form, with tract formation and scar formation. There may be single or multiple widely separated lesions.



Harley stage 3: Diffuse or near-diffuse involvement or multiple interconnected tracts and abscesses are observed across the entire area.



Obstruction (occlusion) of hair follicles and follicular rupture leads 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) that often has an unpleasant odor. 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 HS is unknown, but the condition probably results from a combination of genetic and environmental factors.



Symptoms result from abnormal obstruction (occlusion) of hair follicles and secondary bacterial infection and inflammation, with subsequent rupture of the ducts, spread of infection, and scarring. Another possible cause is abnormal gland development.



Some affected individuals have a family history of the disease, suggesting that genetic factors may play a causative role. . The molecular complex gamma-secretase has been implicated in familial cases of HS.

Affected Populations

HS affects females about three times more often than males. Approximately 1/100 people are affected when mild cases are included. The condition typically becomes apparent during puberty but almost never after the age of about 40.

Standard Therapies

Diagnosis

HS often goes undiagnosed for years because patients are too ashamed to speak with anyone about their symptoms. A diagnosis is made based upon symptoms, medical history, examination and blood tests. If pus or fluid drainage is present, a sample of fluid may be sent for a culture test in order to rule out other skin conditions.



Clinical Testing and Work-Up

Ultrasonography of hair follicles and the thickness of the skin can reveal abnormalities and show how the disease is progressing. Other tests that have been used in evaluating the stage of the disease are C-reactive protein assay, urinalysis, serum IL-2 receptor levels assessment of the erythrocyte sedimentation rate, and a CBC count with differential and platelet counts.



Treatment

There is no known cure for HS. Treatment depends on what clinical stage a patient is at and the severity of their condition. Furthermore, what works for one patient may not work for another. Mild HS can usually be managed with home remedies such as the application of warm compresses, good hygiene, antibacterial soaps, antiseptics, anti-inflammatory medication, and wearing loose-fitting clothing. It is also important for HS patients to maintain a healthy weight and to not smoke. Overweight individuals experience more friction on their body which irritates the skin. They also are more prone to excessive perspiration. Both aid in irritating the skin which causes flare-ups. Smoking also has been shown to be associated more severe disease.



A great number of HS patients have a hard time properly maintaining their weight because exercise causes them pain. Staying healthy is highly important to slowing the progression of the disease, so every effort to find a comfortable exercise routine should be exerted. A lot of patients have reported that swimming not only is great for staying in shape, but provides temporary relief from the painful lesions.



Moderate and severe cases of HS may require medications. Possible medications include antibiotics, oral retinoid medication, anti-inflammatory drugs, corticosteroids, hormones, and tumor necrosis factor-alpha inhibitors. Other treatments that have been shown to be of some benefit are carbon dioxide laser therapy, laser hair removal, radiation therapy, surgery.



Surgical removal of lesions and skin grafting of affected areas may be necessary in cases of extensive, persistent scarred lesions. This surgery is extensive because not only must the skin containing the lesions be removed, but wide margins around the affected area must be removed as well. Surgery has long been considered a cure for this disease, but specific studies have shown otherwise. Ideally, the disease would disappear in the areas that were grafted with new skin; however, recurrence rates can be high particularly if only narrow margins are taken. More limited surgical intervention, consisting of draining abscesses and sinus tracts can be useful for palliation of symptoms but these lesions almost invariably recur.

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

TEXTBOOKS

Jemec, GB (editor). Hidradenitis Suppurativa. 1st ed. New York, NY: Springer-Verlag; 2010.

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



JOURNAL ARTICLES

Jemec, GB. Clinical practice. Hidradenitis suppurativa. N Engl J Med. 2012;366(2):158-64.

Wang, B et al. Gamma-secretase gene mutations in familial acne inversa. Science. 2010;330(6007):1065.



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.



INTERNET

Online Mendelian Inheritance in Man (OMIM). The Johns Hopkins University. Acne Inversa, Familial, 1; ACNINV1. Entry No: 142690. Last Edited December 12, 2011. Available at: http://www.ncbi.nlm.nih.gov/omim/. Accessed September 12, 2012.



Hidradenitis suppurativa. Mayo Clinic. http://www.mayoclinic.com/health/hidradenitis-suppurativa/DS00818. Last updated September 21, 2010. Accessed September 12, 2012.



What is hidradenitis suppurativa? HS-usa.org. http://hs-usa.org//hidradenitis_suppurativa.htm. Accessed September 12, 2012



Fite D. Hidradenitis Suppurativa in Emergency Medicine. Emedicine. http://emedicine.medscape.com/article/762444-overview. Updated July 10, 2012. Accessed September 12, 2012.



Hidradenitis suppurativa. Genetics Home Reference. http://ghr.nlm.nih.gov/condition/hidradenitis-suppurativa. Reviewed March 2010. Published April 16, 2012. Accessed September 12, 2012.

Resources

NIH/National 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

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



Hidradenitis Suppurativa Foundation, Inc

7895 Via Belfiore #4

San Diego, CA 92129

Tel: (858)901-4747

Fax: (619)239-3271

Email: info@hs-foundation.org

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



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.

This information does not replace the advice of a doctor. Healthwise, Incorporated disclaims any warranty or liability for your use of this information. Your use of this information means that you agree to the Terms of Use . How this information was developed to help you make better health decisions.

Healthwise, Healthwise for every health decision, and the Healthwise logo are trademarks of Healthwise, Incorporated.