Steatocystoma Multiplex 

  • Author: Mathew A Davey, MD; Chief Editor: William D James, MD   more...
 
Updated: Jan 12, 2012
 

Background

First described by Jamieson[1] in 1873, and coined by Pringle in 1899, steatocystoma multiplex (SM) is an uncommon disorder of the pilosebaceous unit characterized by the development of numerous sebum-containing dermal cysts. Although steatocystoma multiplex has historically been described as an autosomal dominant inherited disorder, most presenting cases are sporadic.[2]

Steatocystoma simplex is the sporadic solitary tumor counterpart to steatocystoma multiplex.

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Pathophysiology

Steatocystoma multiplex occurs as either a sporadic or autosomal dominant inherited condition characterized by benign sebaceous gland tumors. Lesions consist of a nevoid formation of abortive hair follicles at the site where sebaceous glands attach. Electron microscopy studies demonstrate cyst wall cells undergoing trichilemmal keratinization similar to that of the isthmus portion of the outer hair sheath. The relationship of steatocystoma multiplex to the development of sebaceous glands and common presentation at puberty suggest a hormonal trigger for lesion growth.

In the familial form of steatocystoma multiplex, mutations are localized to the keratin 17 (K17) gene in areas identical to mutations found in patients with pachyonychia congenita type 2 (PC-2). Pachyonychia congenita type 2, an autosomal dominant inherited disorder, is characterized by hypertrophic nail dystrophy, focal keratoderma, multiple pilosebaceous cysts, and a variety of conditions associated with ectodermal dysplasia. Keratin 17 is expressed in several epithelial structures, most notably in sebaceous glands, the outer root sheath of hair follicles, and the nail bed; its expression correlates well to the clinical phenotypic expression of both steatocystoma multiplex and pachyonychia congenita type 2. To date, 14 mutations have been described in patients with either steatocystoma multiplex or pachyonychia congenita type 2, all of which are localized to the helix initiation domain (1A domain) of the K17 gene.[3]

Some authors propose that steatocystoma multiplex is simply a variant of pachyonychia congenita type 2 because they both share the same underlying etiology. Sporadic forms of steatocystoma multiplex have not been shown to be associated with K17 mutations. In previous reports, specific mutations were attributed to early-onset cyst formation in pachyonychia congenita type 2 and steatocystoma multiplex; however, more recent reports suggest that the age of onset is multifactorial.[3]

Steatocystoma multiplex is often associated with eruptive vellus hair cysts (EVHCs). Both diseases share overlapping clinical features, including age of onset, location, appearance of lesions, and mode of inheritance. Reports of hybrid lesions showing histological features of both steatocystoma multiplex and eruptive vellus hair cysts exist.[4] Given these similarities, some postulate that steatocystoma multiplex and eruptive vellus hair cysts are, in fact, variants of the same disease.[2] However, major differences in keratin expression patterns between steatocystoma multiplex and eruptive vellus hair cysts have been elucidated, leading others to believe that they are 2 distinct disease entities.[5] In steatocystoma multiplex associated with eruptive vellus hair cyst, no K17 mutation has been found.

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Epidemiology

Frequency

United States

Steatocystoma multiplex is considered rare; the true incidence is unknown.

Mortality/Morbidity

Steatocystoma multiplex is a benign disorder. In some patients, it may have psychosocial implications resulting from the disfigurement due to widespread lesions or from scarring seen in the inflammatory variant, steatocystoma suppurativa.

Race

No racial predilection has been found.

Sex

Both sexes are equally affected.

Age

In the classic presentation, cysts manifest during adolescence and early adulthood, with average age of onset of 26 years.[2] Cases of steatocystoma multiplex presenting at birth have been reported,[6] and sporadic forms of steatocystoma multiplex with presentation as late as 78 years have been described.[7] Once present, steatocystoma multiplex is a lifelong condition.

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Contributor Information and Disclosures
Author

Mathew A Davey, MD  Resident Physician, Department of Dermatology, University of North Carolina at Chapel Hill

Mathew A Davey, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and American Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

Craig N Burkhart, MD, MSBS  Assistant Professor, Department of Dermatology, University of North Carolina at Chapel Hill

Craig N Burkhart, MD, MSBS is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, and American Medical Association

Disclosure: Nothing to disclose.

Dean Scott Morrell, MD  Associate Professor, Director of Residency Training Program, Director of Pediatric and Adolescent Dermatology, Department of Dermatology, University of North Carolina at Chapel Hill

Disclosure: Nothing to disclose.

Specialty Editor Board

Evan R Farmer, MD  Clinical Professor of Pathology and Dermatology, Department of Pathology, Virginia Commonwealth University School of Medicine

Evan R Farmer, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Medical Association, American Society of Dermatopathology, and International Society of Dermatology

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Warren R Heymann, MD  Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

William D James, MD  Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology

Disclosure: Elsevier Royalty Other

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Mary Bane, MD, to the development and writing of this article.

References

  1. Jamieson WA. Case of numerous cutaneous cysts scattered over the body. Edin Med J. 1873;19:223-5.

  2. Cho S, Chang SE, Choi JH, Sung KJ, Moon KC, Koh JK. Clinical and histologic features of 64 cases of steatocystoma multiplex. J Dermatol. Mar 2002;29(3):152-6. [Medline].

  3. Oh SW, Kim MY, Lee JS, Kim SC. Keratin 17 mutation in pachyonychia congenita type 2 patient with early onset steatocystoma multiplex and Hutchinson-like tooth deformity. J Dermatol. Mar 2006;33(3):161-4. [Medline].

  4. Yamada A, Saga K, Jimbow K. Acquired multiple pilosebaceous cysts on the face having the histopathological features of steatocystoma multiplex and eruptive vellus hair cysts. Int J Dermatol. Oct 2005;44(10):861-3. [Medline].

  5. Tomková H, Fujimoto W, Arata J. Expression of keratins (K10 and K17) in steatocystoma multiplex, eruptive vellus hair cysts, and epidermoid and trichilemmal cysts. Am J Dermatopathol. Jun 1997;19(3):250-3. [Medline].

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  8. Mortazavi H, Taheri A, Mansoori P, Kani ZA. Localized forms of steatocystoma multiplex: Case report and review of the literature. Dermatology Online Journal [serial online]. 2005;11:22. Available from: http://dermatology.cdlib.org/. Accessed October 21, 2008. Available at http://dermatology-s10.cdlib.org/111/case_presentations/steatocystoma/taheri.html.

  9. Lee D, Chun JS, Hong SK, Seo JK, Choi JH, Koh JK, et al. Steatocystoma multiplex confined to the scalp with concurrent alopecia. Ann Dermatol. Oct 2011;23:S258-60. [Medline]. [Full Text].

  10. Rollins T, Levin RM, Heymann WR. Acral steatocystoma multiplex. J Am Acad Dermatol. Aug 2000;43(2 Pt 2):396-9. [Medline].

  11. Duzova AN, Senturk GB. Suggestion for the treatment of steatocystoma multiplex located exclusively on the face. Int J Dermatol. Jan 2004;43(1):60-2. [Medline].

  12. Schmook T, Burg G, Hafner J. Surgical pearl: mini-incisions for the extraction of steatocystoma multiplex. J Am Acad Dermatol. Jun 2001;44(6):1041-2. [Medline].

  13. Kaya TI, Ikizoglu G, Kokturk A, Tursen U. A simple surgical technique for the treatment of steatocystoma multiplex. Int J Dermatol. Dec 2001;40(12):785-8. [Medline].

  14. Lee SJ, Choe YS, Park BC, Lee WJ, Kim do W. The vein hook successfully used for eradication of steatocystoma multiplex. Dermatologic Surgery. 2008;33:82-84.

  15. Choudhary S, Koley S, Salodkar A. A modified surgical technique for steatocystoma multiplex. J Cutan Aesthet Surg. Jan 2010;3(1):25-8. [Medline]. [Full Text].

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  17. Adams BB, Mutasim DF, Nordlund JJ. Steatocystoma multiplex: a quick removal technique. Cutis. Aug 1999;64(2):127-30. [Medline].

  18. Brownstein MH. Steatocystoma simplex. A solitary steatocystoma. Arch Dermatol. Jun 1982;118(6):409-11. [Medline].

  19. Chu, D. H. Steatocystoma multiplex. Dermatology Online Journal [serial online]. 2003;9:18. Available from: http://dermatology.cdlib.org/. Accessed October 21, 2008. Available at http://dermatology.cdlib.org/94/NYU/Feb2002/7.html.

  20. Corden LD, McLean WH. Human keratin diseases: hereditary fragility of specific epithelial tissues. Exp Dermatol. Dec 1996;5(6):297-307. [Medline].

  21. Kaur T, Kanwar AJ. Steatocystoma multiplex in four successive generations. J Dermatol. Jul 2003;30(7):559-61. [Medline].

  22. Kligman AM, Kirschbaum JD. Steatocystoma multiplex: a dermoid tumor. J Invest Dermatol. May 1964;42:383-7. [Medline].

  23. Krahenbuhl A, Eichmann A, Pfaltz M. CO2 laser therapy for steatocystoma multiplex. Dermatologica. 1991;183(4):294-6. [Medline].

  24. Laquer VT, Wu JJ, Tournas JA, Murase JE, Dyson SW. Pruritic bluish-black subcutaneous papules on the chest. Dermatology Online Journal [serial online]. 2008;14:14. Available from: http://dermatology.cdlib.org/. Accessed October 21, 2008. Available at http://dermatology-s10.cdlib.org/143/case_presentations/steatocystoma/wu.html.

  25. Plewig G, Wolff HH, Braun-Falco O. Steatocystoma multiplex: anatomic reevaluation, electron microscopy, and autoradiography. Arch Dermatol Res. 1982;272(3-4):363-80. [Medline].

  26. Smith FJ, Corden LD, Rugg EL, et al. Missense mutations in keratin 17 cause either pachyonychia congenita type 2 or a phenotype resembling steatocystoma multiplex. J Invest Dermatol. Feb 1997;108(2):220-3. [Medline].

 
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Steatocystoma multiplex on the chest of an adolescent female.
Steatocystoma multiplex with typical-appearing, smooth, yellow and white dermal cysts.
Note the crenulated eosinophilic lining of the cyst wall (10X magnification).
Note the sebaceous glands within the cyst wall (2X scanning view).
 
 
 
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