eMedicine Specialties > Dermatology > Benign Neoplasms

Steatocystoma Multiplex

Author: Mathew A Davey, MD, Resident Physician, Department of Dermatology, University of North Carolina at Chapel Hill
Coauthor(s): Craig N Burkhart, MD, MSBS, Assistant Professor, Department of Dermatology, University of North Carolina at Chapel Hill; 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
Contributor Information and Disclosures

Updated: Apr 14, 2009

Introduction

Background

First described by Jamieson1 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.

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.

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.

Clinical

History

Affected individuals often present with an increasing number of smooth flesh-to-yellow–colored cysts. The cysts are usually nontender and asymptomatic. On occasion, individual lesions may rupture into the dermis, become inflamed, and form sinus tracts with scarring. Secondary bacterial colonization can lead to malodorous discharge.

Physical

Lesions present as numerous flesh-to-yellow–colored dermal cysts ranging in size from 3 mm to 3 cm. Individual cysts range from elastic to firm and are often freely movable. The lesions lack a central punctum. Cyst contents appear as an odorless creamy or oily fluid. Individual lesions of steatocystoma multiplex may become suppurative, increase in size, and become prone to rupture (termed steatocystoma multiplex suppurativum). In these cases, secondary bacterial colonization often leads to malodorous discharge. Significant scarring with sinus tract formation may occur.

In typical cases of steatocystoma multiplex, cysts are distributed in areas where high numbers of sebaceous glands are found, most commonly the chest, arms, axillae, and neck. Several reports of localized steatocystoma multiplex limited to the scalp, face, retroauricular region, groin, and nasal region have been reported.8 Acral steatocystoma multiplex, in which involvement of the extremities is more prominent than the trunk, is uncommon and was described by Rollins et al in 2000.9

Steatocystoma multiplex on the chest of an adoles...

Steatocystoma multiplex on the chest of an adolescent female.

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Steatocystoma multiplex on the chest of an adoles...

Steatocystoma multiplex on the chest of an adolescent female.


Steatocystoma multiplex with typical-appearing, s...

Steatocystoma multiplex with typical-appearing, smooth, yellow and white dermal cysts.

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Steatocystoma multiplex with typical-appearing, s...

Steatocystoma multiplex with typical-appearing, smooth, yellow and white dermal cysts.


While some authors refer to localized steatocystoma multiplex as a specific condition, it shares pathological and clinical features of typical cases and is thought to represent a variant of the steatocystoma multiplex rather than a separate disease entity.8 Linear variants have been reported,6 and, although rare, generalized eruptions may occur.

Causes

Steatocystoma multiplex is a disorder of the pilosebaceous unit that occurs in either a sporadic or an autosomal dominant fashion. Androgenic stimulation of the sebaceous gland, along with environmental factors and the site and type of the keratin mutation, influence the onset of the sebaceous cysts.3

More on Steatocystoma Multiplex

Overview: Steatocystoma Multiplex
Differential Diagnoses & Workup: Steatocystoma Multiplex
Treatment & Medication: Steatocystoma Multiplex
Follow-up: Steatocystoma Multiplex
Multimedia: Steatocystoma Multiplex
References
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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].

  6. Park YM, Cho SH, Kang H. Congenital linear steatocystoma multiplex of the nose. Pediatr Dermatol. Mar-Apr 2000;17(2):136-8. [Medline].

  7. Riedel C, Brinkmeier T, Kutzne H, Plewig G, Frosch PJ. Late onset of a facial variant of steatocystoma multiplex - calretinin as a specific marker of the follicular companion cell layer. J Dtsch Dermatol Ges. Jun 2008;6(6):480-2. [Medline].

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

  10. 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].

  11. 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].

  12. 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].

  13. 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.

  14. Rossi R, Cappugi P, Battini M, Mavilia L, Campolmi P. CO2 laser therapy in a case of steatocystoma multiplex with prominent nodules on the face and neck. Int J Dermatol. Apr 2003;42(4):302-4. [Medline].

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

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

  17. 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.

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

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

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

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

  22. 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.

  23. 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].

  24. 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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Further Reading

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Keywords

steatocystoma multiplex, sebocystomatosis, hereditary epidermal polycystic disease

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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.

Medical Editor

Evan R Farmer, MD, Professor of Dermatology, Johns Hopkins University School of Medicine, Clinical Professor of Pathology, Virginia Commonwealth University School of Medicine; Consulting Staff, Department of Dermatology, Johns Hopkins Hospital, VCU Health Services
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.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University 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.

Managing Editor

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

CME Editor

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 None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

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