Steatocystoma Multiplex
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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.
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 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, 5] 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. [6] In steatocystoma multiplex associated with eruptive vellus hair cyst, no K17 mutation has been found.
Steatocystoma multiplex is considered rare; the true incidence is unknown.
No racial predilection has been found.
Both sexes are equally affected.
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, [7] and sporadic forms of steatocystoma multiplex with presentation as late as 78 years have been described. [8] Once present, steatocystoma multiplex is a lifelong condition.
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. The prognosis for patients with steatocystoma multiplex is excellent. No reports describe malignant transformation within these benign adnexal tumors.
Jamieson WA. Case of numerous cutaneous cysts scattered over the body. Edin Med J. 1873. 19:223-5.
Cho S, Chang SE, Choi JH, Sung KJ, Moon KC, Koh JK. Clinical and histologic features of 64 cases of steatocystoma multiplex. J Dermatol. 2002 Mar. 29(3):152-6. [Medline].
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. 2006 Mar. 33(3):161-4. [Medline].
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. 2005 Oct. 44(10):861-3. [Medline].
Papakonstantinou E, Franke I, Gollnick H. Facial steatocystoma multiplex combined with eruptive vellus hair cysts: a hybrid?. J Eur Acad Dermatol Venereol. 2014 Jul 30. [Medline].
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. 1997 Jun. 19(3):250-3. [Medline].
Park YM, Cho SH, Kang H. Congenital linear steatocystoma multiplex of the nose. Pediatr Dermatol. 2000 Mar-Apr. 17(2):136-8. [Medline].
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. 2008 Jun. 6(6):480-2. [Medline].
Mortazavi H, Taheri A, Mansoori P, Kani ZA. Localized forms of steatocystoma multiplex: Case report and review of the literature. Dermatology Online Journal. 2005. 11:22. [Full Text].
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. 2011 Oct. 23:S258-60. [Medline]. [Full Text].
Rollins T, Levin RM, Heymann WR. Acral steatocystoma multiplex. J Am Acad Dermatol. 2000 Aug. 43(2 Pt 2):396-9. [Medline].
Duzova AN, Senturk GB. Suggestion for the treatment of steatocystoma multiplex located exclusively on the face. Int J Dermatol. 2004 Jan. 43(1):60-2. [Medline].
Schmook T, Burg G, Hafner J. Surgical pearl: mini-incisions for the extraction of steatocystoma multiplex. J Am Acad Dermatol. 2001 Jun. 44(6):1041-2. [Medline].
Kaya TI, Ikizoglu G, Kokturk A, Tursen U. A simple surgical technique for the treatment of steatocystoma multiplex. Int J Dermatol. 2001 Dec. 40(12):785-8. [Medline].
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.
Choudhary S, Koley S, Salodkar A. A modified surgical technique for steatocystoma multiplex. J Cutan Aesthet Surg. 2010 Jan. 3(1):25-8. [Medline]. [Full Text].
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. 2003 Apr. 42(4):302-4. [Medline].
Krahenbuhl A, Eichmann A, Pfaltz M. CO2 laser therapy for steatocystoma multiplex. Dermatologica. 1991. 183(4):294-6. [Medline].
Bakkour W, Madan V. Carbon dioxide laser perforation and extirpation of steatocystoma multiplex. Dermatol Surg. 2014 Jun. 40(6):658-62. [Medline].
Dean Scott Morrell, MD Professor, Director of Dermatology Residency Training Program, Director of Pediatric and Adolescent Dermatology, Department of Dermatology, University of North Carolina at Chapel Hill
Disclosure: Nothing to disclose.
Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Received salary from Medscape for employment. for: Medscape.
Steven R Feldman, MD, PhD Professor, Departments of Dermatology, Pathology and Public Health Sciences, and Molecular Medicine and Translational Science, Wake Forest Baptist Health; Director, Center for Dermatology Research, Director of Industry Relations, Department of Dermatology, Wake Forest University School of Medicine
Steven R Feldman, MD, PhD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, North Carolina Medical Society, Society for Investigative Dermatology
Disclosure: Received honoraria from Amgen for consulting; Received honoraria from Abbvie for consulting; Received honoraria from Galderma for speaking and teaching; Received consulting fee from Lilly for consulting; Received ownership interest from www.DrScore.com for management position; Received ownership interest from Causa Reseasrch for management position; Received grant/research funds from Janssen for consulting; Received honoraria from Pfizer for speaking and teaching; Received consulting fee from No.
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, Society for Investigative Dermatology
Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD.
Craig N Burkhart, MD, MSBS Assistant Professor, Department of Dermatology, University of North Carolina at Chapel Hill School of Medicine
Craig N Burkhart, MD, MSBS is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Medical Association
Disclosure: Nothing to disclose.
Mathew A Davey, MD, FAAD Dermatologist, Advanced Dermatology of the Midlands
Mathew A Davey, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association
Disclosure: Nothing to disclose.
Arash Taheri, MD Research Fellow, Center for Dermatology Research, Department of Dermatology, Wake Forest University School of Medicine
Disclosure: Nothing to disclose.
The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Mary Bane, MD, to the development and writing of this article.
Steatocystoma Multiplex
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