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Genetics Home Reference: your guide to understanding genetic conditions
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Hidradenitis suppurativa

Reviewed March 2010

What is hidradenitis suppurativa?

Hidradenitis suppurativa is a chronic skin disease characterized by recurrent boil-like lumps (nodules) under the skin. The nodules are inflamed and painful. They tend to break open, causing abscesses that drain fluid and pus. As the abscesses heal, they produce significant scarring of the skin.

Nodules are most likely to form in the armpits and groin. They may also develop around the anus, on the buttocks, or under the breasts. In some cases, nodules appear in other areas, such as the nape of the neck, waist, and inner thighs.

The signs and symptoms of hidradenitis suppurativa appear after puberty, usually in a person's teens or twenties.

The recurrent nodules and abscesses cause chronic pain and can lead to self-consciousness, social isolation, and depression. Less common complications of hidradenitis suppurativa include severe infections, restricted movement caused by a buildup of scar tissue (fibrosis), and a type of skin cancer called squamous cell carcinoma.

How common is hidradenitis suppurativa?

Hidradenitis suppurativa was once thought to be a rare condition because only the most severe cases were reported. However, recent studies have shown that the condition affects about 1 in 100 people when milder cases are also considered. For reasons that are unclear, women are about three times more likely than men to develop the condition.

What genes are related to hidradenitis suppurativa?

The cause of hidradenitis suppurativa is unknown, although it probably results from a combination of genetic and environmental factors. Originally, researchers believed that the disorder was caused by the blockage of specialized sweat glands called apocrine glands. However, recent studies have shown that the condition actually begins with a blockage of hair follicles in areas of the body that also contain a high concentration of apocrine glands (such as the armpits and groin). The blocked hair follicles trap bacteria, leading to infection and inflammation. It remains unclear what initially causes the follicles to become blocked and why the nodules tend to recur.

Researchers have studied many possible risk factors for hidradenitis suppurativa. Obesity and smoking both appear to increase the risk of the disorder, and obesity is also associated with increased severity of signs and symptoms in affected individuals. Studies suggest that neither abnormal immune system function nor hormonal factors play a significant role in causing the disease. Other factors that were mistakenly thought to be associated with this condition include poor hygiene, the use of underarm deodorants and antiperspirants, and shaving or the use of depilatory products to remove hair.

Genetic factors clearly play a role in causing hidradenitis suppurativa. In one large family, researchers determined that the condition was related to a gene on chromosome 1 (although they were not able to identify the specific gene). Researchers are looking for additional genetic factors that are associated with hidradenitis suppurativa.

How do people inherit hidradenitis suppurativa?

Hidradenitis suppurativa has been reported to run in families. Studies have found that 30 to 40 percent of affected individuals have at least one family member with the disorder, although this finding may be an underestimate because affected individuals do not always tell their family members that they have the condition.

In some families, hidradenitis suppurativa appears to have an autosomal dominant pattern of inheritance, which means one copy of an altered gene in each cell is sufficient to cause the disorder. However, the specific gene or genes have not been identified.

Where can I find information about diagnosis or management of hidradenitis suppurativa?

These resources address the diagnosis or management of hidradenitis suppurativa and may include treatment providers.

  • Genetic Testing Registry: Hidradenitis suppurativa, familial (http://www.ncbi.nlm.nih.gov/gtr/conditions/C1840560)

You might also find information on the diagnosis or management of hidradenitis suppurativa in Educational resources (http://www.ghr.nlm.nih.gov/condition/hidradenitis-suppurativa/show/Educational+resources) and Patient support (http://www.ghr.nlm.nih.gov/condition/hidradenitis-suppurativa/show/Patient+support).

General information about the diagnosis (http://ghr.nlm.nih.gov/handbook/consult/diagnosis) and management (http://ghr.nlm.nih.gov/handbook/consult/treatment) of genetic conditions is available in the Handbook. Read more about genetic testing (http://ghr.nlm.nih.gov/handbook/testing), particularly the difference between clinical tests and research tests (http://ghr.nlm.nih.gov/handbook/testing/researchtesting).

To locate a healthcare provider, see How can I find a genetics professional in my area? (http://ghr.nlm.nih.gov/handbook/consult/findingprofessional) in the Handbook.

Where can I find additional information about hidradenitis suppurativa?

You may find the following resources about hidradenitis suppurativa helpful. These materials are written for the general public.

You may also be interested in these resources, which are designed for healthcare professionals and researchers.

What other names do people use for hidradenitis suppurativa?

  • acne inversa
  • hidradenitides, suppurative
  • hidradenitis, suppurative
  • suppurative hidradenitides
  • suppurative hidradenitis

For more information about naming genetic conditions, see the Genetics Home Reference Condition Naming Guidelines (http://ghr.nlm.nih.gov/ConditionNameGuide) and How are genetic conditions and genes named? (http://ghr.nlm.nih.gov/handbook/mutationsanddisorders/naming) in the Handbook.

What if I still have specific questions about hidradenitis suppurativa?

Ask the Genetic and Rare Diseases Information Center (http://rarediseases.info.nih.gov/GARD/).

What glossary definitions help with understanding hidradenitis suppurativa?

acne ; anus ; autosomal ; autosomal dominant ; bacteria ; cancer ; carcinoma ; cell ; chromosome ; chronic ; depression ; fibrosis ; gene ; groin ; immune system ; infection ; inflammation ; inheritance ; pattern of inheritance ; puberty ; risk factors ; tissue

You may find definitions for these and many other terms in the Genetics Home Reference Glossary (http://www.ghr.nlm.nih.gov/glossary).

References

  • Alikhan A, Lynch PJ, Eisen DB. Hidradenitis suppurativa: a comprehensive review. J Am Acad Dermatol. 2009 Apr;60(4):539-61; quiz 562-3. doi: 10.1016/j.jaad.2008.11.911. Review. (http://www.ncbi.nlm.nih.gov/pubmed/19293006?dopt=Abstract)
  • Buimer MG, Wobbes T, Klinkenbijl JH. Hidradenitis suppurativa. Br J Surg. 2009 Apr;96(4):350-60. doi: 10.1002/bjs.6569. Review. (http://www.ncbi.nlm.nih.gov/pubmed/19283748?dopt=Abstract)
  • Canoui-Poitrine F, Revuz JE, Wolkenstein P, Viallette C, Gabison G, Pouget F, Poli F, Faye O, Bastuji-Garin S. Clinical characteristics of a series of 302 French patients with hidradenitis suppurativa, with an analysis of factors associated with disease severity. J Am Acad Dermatol. 2009 Jul;61(1):51-7. doi: 10.1016/j.jaad.2009.02.013. Epub 2009 Apr 29. (http://www.ncbi.nlm.nih.gov/pubmed/19406505?dopt=Abstract)
  • Gao M, Wang PG, Cui Y, Yang S, Zhang YH, Lin D, Zhang KY, Liang YH, Sun LD, Yan KL, Xiao FL, Huang W, Zhang XJ. Inversa acne (hidradenitis suppurativa): a case report and identification of the locus at chromosome 1p21.1-1q25.3. J Invest Dermatol. 2006 Jun;126(6):1302-6. (http://www.ncbi.nlm.nih.gov/pubmed/16543891?dopt=Abstract)
  • Jemec GB. Hidradenitis suppurativa. J Cutan Med Surg. 2003 Jan-Feb;7(1):47-56. Epub 2002 Dec 4. Review. (http://www.ncbi.nlm.nih.gov/pubmed/12457209?dopt=Abstract)
  • Revuz J. Hidradenitis suppurativa. J Eur Acad Dermatol Venereol. 2009 Sep;23(9):985-98. doi: 10.1111/j.1468-3083.2009.03356.x. (http://www.ncbi.nlm.nih.gov/pubmed/19682181?dopt=Abstract)
  • Revuz JE, Canoui-Poitrine F, Wolkenstein P, Viallette C, Gabison G, Pouget F, Poli F, Faye O, Roujeau JC, Bonnelye G, Grob JJ, Bastuji-Garin S. Prevalence and factors associated with hidradenitis suppurativa: results from two case-control studies. J Am Acad Dermatol. 2008 Oct;59(4):596-601. doi: 10.1016/j.jaad.2008.06.020. (http://www.ncbi.nlm.nih.gov/pubmed/18674845?dopt=Abstract)
  • von der Werth JM, Jemec GB. Morbidity in patients with hidradenitis suppurativa. Br J Dermatol. 2001 Apr;144(4):809-13. (http://www.ncbi.nlm.nih.gov/pubmed/11298541?dopt=Abstract)
  • Von Der Werth JM, Williams HC, Raeburn JA. The clinical genetics of hidradenitis suppurativa revisited. Br J Dermatol. 2000 May;142(5):947-53. (http://www.ncbi.nlm.nih.gov/pubmed/10809853?dopt=Abstract)

 

The resources on this site should not be used as a substitute for professional medical care or advice. Users seeking information about a personal genetic disease, syndrome, or condition should consult with a qualified healthcare professional. See How can I find a genetics professional in my area? (http://ghr.nlm.nih.gov/handbook/consult/findingprofessional) in the Handbook.

 
Reviewed: March 2010
Published: June 17, 2013