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Steatocystoma multiplex


Other Names for this Disease

  • Multiple sebaceous cysts
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

What is steatocystoma multiplex?

What are the signs and symptoms of steatocystoma multiplex?

What causes steatocystoma multiplex?

How might steatocystoma multiplex be treated?

What is steatocystoma multiplex?

Steatocystoma multiplex is a condition characterized by numerous skin cysts that tend to develop during puberty. Cysts most often develop on the chest, upper arms and face, but may develop all over the body in some cases. The cysts may become inflamed and cause scarring when they heal. The condition is thought to be caused by mutations in the KRT17 gene and appears to be inherited in an autosomal dominant manner.[1][2] Some researchers have suggested that the condition may be a mild variant of pachyonychia congenita type 2.[3] Treatment may include minor surgery to remove cysts and oral antibiotics or oral isotretinoin to reduce inflammation.[1]
Last updated: 6/6/2012

What are the signs and symptoms of steatocystoma multiplex?

Signs and symptoms of steatocystoma multiplex include multiple cysts on the skin. The cysts are often 1 to 2 centimeter wide. They frequently occur on the trunk of the body, upper arms, legs, and face; however, they can develop on other parts of the body as well.[4][5][1]The cysts are typically filled with a yellowish to white, oily fluid, and occasionally have hair within them. The cysts can become infected and may cause pain and scarring.[6]
Last updated: 6/8/2012

What causes steatocystoma multiplex?

Mutations in a gene called keratin 17 (KRT17) have been identified in some individuals with inherited steatocystoma multiplex. In these families the condition is inherited in an autosomal dominant fashion.[7][8][9]

In other cases the condition occurs sporadically. This may mean that it is due to a gene mutation that was not inherited, but occurred for the first time in the affected individual. A sporadic condition may also be non-genetic and occur by chance, in which case it is not likely to recur in a family.  In many sporadic cases of steatocystoma multiplex, mutations in the KRT17 gene have not been identified.[7]

Cases of steatocystoma multiplex have also been reported in association with pachyonychia congenita, acrokeratosis verruciformis, hypertrophic lichen planus, hypohidrosis, hidradenitis suppurativa, and natal teeth.[4]

Last updated: 6/8/2012

How might steatocystoma multiplex be treated?

Treatment options for steatocystoma multiplex are limited and have had varying degrees of success. The most effective treatment method is thought to be removal of cysts by surgery.  However, cosmetic concerns, time, cost, and pain need to be considered because affected individuals often have multiple cysts.[10]  In many cases, small incisions (cuts into the skin) allow the cyst and its contents to be removed through the opening.[1]  Other treatment options include medications such as oral isotretinoin to temporarily shrink the cysts and reduce inflammation or oral antibiotics to reduce redness and swelling.  Other procedures may include draining cysts through a procedure called aspirationliquid nitrogen cryotherapydermabrasion, and carbon dioxide laser therapy.[1][10][6][11]

In a recently published case study, the authors present a case in which an individual with steatocystomas on the abdomen and lower chest showed substantial clearance of cysts after two laser treatment sessions.[10] Future studies with a larger patient population will be helpful to evaluate this noninvasive treatment option and determine ideal treatment settings, number of treatments, and interval between treatments. This may prove to be an option for individuals with numerous cysts, in whom removal and drainage is not a realistic choice and other treatments have failed to improve the condition.[10]
Last updated: 6/23/2014

References
  1. Amanda Oakley. Steatocystoma multiplex. DermNet NZ. February 22, 2014; http://www.dermnetnz.org/lesions/steatocystoma-multiplex.html. Accessed 6/23/2014.
  2. Victor A. McKusick. Steatocystoma Multiplex. OMIM. June 9, 1997; http://omim.org/entry/184500. Accessed 6/6/2012.
  3. KRT17. Genetics Home Reference. August 2008; http://ghr.nlm.nih.gov/gene/KRT17. Accessed 6/6/2012.
  4. Naik NS. Steatocystoma multiplex. Dermatol Online J. 2000 Sep;
  5. Steatocystoma-Multiplex. New Zealand Dermatologic Society, Inc. 1999; http://www.dermnetnz.org/lesions/steatocystoma-multiplex.html. Accessed 11/10/2009.
  6. Lee KK, Mehrany K, Swanson NA. Recognition and Treatment of Skin Lesions. In: Cummings. Otolaryngology: Head & Neck Surgery, 4th ed. Philadelphia, PA: Mosby, Inc; 2005;
  7. Chu DH. Steatocystoma multiplex. Dermatol Online J. 2003 Oct;
  8. Pamoukian VN, Westreich M. Five generations with steatocystoma multiplex congenita: a treatment regimen. Plast Reconst Surg. 1997;
  9. Steatocystoma multiplex. Online Mendialian Inheritance in Man. 1997; http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=184500. Accessed 11/10/2009.
  10. Moody MN, Landau JM, Goldberg LH, Friedman PM. 1,450-nm Diode Laser in Combination with the 1550-nm Fractionated Erbium-Doped Fiber Laser for the Treatment of Steatocystoma Multiplex: A Case Report. Dermatol Surg. April 2012; Epub:
  11. Davey, MA. Steatocystoma Multiplex Treatment & Management. Medscape. October 26, 2012; http://emedicine.medscape.com/article/1059725-treatment. Accessed 6/23/2014.


Other Names for this Disease
  • Multiple sebaceous cysts
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.