Photo Essay

An Atlas of Lumps and Bumps, Part 45: Steatocystoma Multiplex

Alexander K.C. Leung, MD, MBBS, FRCPC, FRCP
Benjamin Barankin, MD, FRCPC
Joseph M. Lam, MD
Kin Fon Leong, MD
Volume 64 - Issue 11 - November 2024

Steatocystoma Multiplex 

Steatocystoma multiplex is a hamartomatous malformation of the pilosebaceous glands characterized by multiple skin-colored sebum-containing dermal cystic papules and nodules.1-3 The condition frequently presents in adolescence and early adulthood.3-6 Occasionally, it can appear earlier only to worsen at puberty, when the activity of the sebaceous glands is elevated.4 Steatocystoma multiplex has been described at birth and in patients in their eighth decade.2,3,7  There is no sex predilection.2,6,8

Steatocystoma multiplex is a rare skin condition; the exact incidence is not known. Familial cases are often caused by mutations in exon 1 of the keratin 17 (KRT17) gene located on chromosome 17q21.2 and have an autosomal dominant mode of inheritance.9-12 The majority of cases are sporadic.8,13,14 Androgenic stimulation of the sebaceous glands, genetic predisposition, along with environmental factors influence the onset of steatocystoma multiplex. The condition has been reported following the use of ustekinumab which might have unmasked a genetic predisposition.15 Hypertrophic lichen planus, eruptive vellus hair cysts, pachyonychia congenita type 2 (Jackson-Lawler syndrome), hypohidrosis, congenital alopecia, acrokeratosis verruciformis, hidradenitis suppurativa, hypothyroidism, preauricular sinuses, and natal teeth occur with increased frequently in patients with steatocystoma multiplex.5,16-19

Clinically, steatocystoma multiplex is characterized by multiple, asymptomatic, smooth, round, soft to firm, often freely movable, yellow to skin-colored papules and nodules (Figure 1).1,7,8


Figure 1. Steatocystoma multiplex is characterized by multiple, asymptomatic, smooth, round, soft to firm, often freely movable, yellow, skin-colored papules and nodules.

Lesions tend to be a few millimeters to a centimeter in diameter and are slow growing.1 Superficial lesions are usually yellowish while deeper lesions skin-colored (Figure 2).1,8 The overlying epidermis is normal with no central punctum present. 1,8,20 The content is usually oily or creamy. Sites of predilection include the trunk (especially the presternal area), neck, axillae, proximal extremities, and groin where high numbers of sebaceous glands are found.1,4,7,21


Figure 2. Sites of predilection for steatocystoma multiplex include the trunk, particularly the presternal area, as well as the neck, axillae, proximal extremities, and groin where high numbers of sebaceous glands are found.

Rarely, the face, scalp, breasts, and genital area are affected.1,2,9,20-22 There is usually little or no nail or hair involvement. There are no systemic manifestations.1,14

Several variants exist. In the suppurative variant, the lesions become inflamed and suppurative after minor trauma.14 In the acral variant, the lesions appear on the distal upper or lower extremities.7,15 The female to male ratio is 4:1 in the acral variant.3 Localized forms of steatocystoma multiplex have also been described, such as cases with a cephalic or genital localization.23

The diagnosis is usually clinical, based on typical physical findings. There are no abnormal laboratory findings. Skin biopsy should be considered if the diagnosis is in doubt.

Steatocystoma multiplex is cosmetically unsightly and may have an adverse impact on quality of life.18 Occasionally, the lesions may rupture, become infected and painful, and heal with scarring.6,9,24 Rarely, the lesions may calcify.8 Malignant degeneration has rarely been described.25 Without treatment, the lesion tends to persist and may slowly enlarge.

AUTHORS:
Alexander K.C. Leung, MD1,2, Benjamin Barankin, MD3, Joseph M. Lam, MD4, Kin Fon Leong, MD5

AFFILIATIONS:
1Clinical Professor of Pediatrics, the University of Calgary, Calgary, Alberta, Canada
2Pediatric Consultant, the Alberta Children’s Hospital, Calgary, Alberta, Canada
3Dermatologist, Medical Director and Founder, the Toronto Dermatology Centre, Toronto, Ontario, Canada
4Associate Clinical Professor of Pediatrics, Dermatology and Skin Sciences, the University of British Columbia, Vancouver, British Columbia, Canada.
5Pediatric Dermatologist, the Pediatric Institute, Kuala Lumpur General Hospital, Kuala Lumpur, Malaysia

CITATION:
Leung AKC, Barankin B, Lam JM, Leong KF. An Atlas of Lumps and Bumps, Part 44: Dermatosis papulosa nigra. Consultant. 2024;64(10):eXX. doi:10.25270/con.2024.10.000005

CORRESPONDENCE:
Alexander K. C. Leung, MD, #200, 233 16th Ave NW, Calgary, AB T2M 0H5, Canada (aleung@ucalgary.ca)

EDITOR’S NOTE:
This article is part of a series describing and differentiating dermatologic lumps and bumps. To access previously published articles in the series, visit: https://www.consultant360.com/resource-center/atlas-lumps-and-bumps.

References

  1. Leung AKC, Barankin B. A man with a 15-year history of asymptomatic dermal cysts on the abdomen: Steatocystoma multiplex. Consultant. 2016;56:609-610.
  2. Jiang M, Zhang M, Gu H, Chen X. A case of late onset steatocystoma multiplex. Postepy Dermatol Alergol. 2020;37(1):117-118. doi: 10.5114/ada.2018.78999.
  3. Marzano AV, Tavecchio S, Balice Y, Polloni I, Veraldi S. Acral subcutaneous steatocystoma multiplex: a distinct subtype of the disease? Australas J Dermatol. 2012;53(3):198-201. doi: 10.1111/j.1440-0960.2011.00851.x.
  4. Bakkour W, Madan V. Carbon dioxide laser perforation and extirpation of steatocystoma multiplex. Dermatol Surg. 2014;40(6):658-662. doi: 10.1111/dsu.0000000000000013.
  5. Chotai N, Lim SK. Imaging features of steatocystoma multiplex- back to basics. Breast J. 2021 Feb 1. doi: 10.1111/tbj.14179. Online ahead of print.
  6. Santana CN, Pereira DD, Lisboa AP, Leal JM, Obadia DL, Silva RS. Steatocystoma multiplex suppurativa: case report of a rare condition. An Bras Dermatol. 2016;91(5 suppl 1):51-53. doi: 10.1590/abd1806-4841.20164539.
  7. Rollins T, Levin RM, Heymann WR. Acral steatocystoma multiplex. J Am Acad Dermatol. 2000;43(2 Pt 2):396-399. doi: 10.1067/mjd.2000.100048.
  8. Rahman MH, Islam MS, Ansari NP. Atypical steatocystoma multiplex with calcification. ISRN Dermatology. 2011;2011:381901. doi: 10.5402/2011/381901.
  9. Alotaibi L, Alsaif M, Alhumidi A, Turkmani M, Alsaif F. Steatocystoma multiplex suppurativa: A case with unusual giant cysts over the scalp and neck. Case Rep Dermatol. 2019;11(1):71-76. doi: 10.1159/000498882.
  10. Liu Q, Wu W, Lu J, Wang P, Qiao F. Steatocystoma multiplex is associated with the R94C mutation in the KRTl7 gene. Mol Med Rep. 2015;12(4):5072-5076. doi: 10.3892/mmr.2015.4063.
  11. Wang J, Li J, Li X, et al. A recurrent mutation in the KRT17 gene responsible for severe steatocystoma multiplex in a large Chinese family. Clin Exp Dermatol. 2018;43(2):205-208. doi: 10.1111/ced.13311.
  12. Zhang B, Sun L, Fu X, Yu G, Liu H, Zhang F. Mutation analysis of the KRT17 gene in steatocystoma multiplex and a brief literature review. Clin Exp Dermatol. 2020;45(1):132-134. doi: 10.1111/ced.14030.
  13. Georgakopoulos JR, Ighani A, Yeung J. Numerous asymptomatic dermal cysts: Diagnosis and treatment of steatocystoma multiplex. Can Fam Physician. 2018;64(12):892-899.
  14. Gordon Spratt EA, Kaplan J, Patel RR, Kamino H, Ramachandran SM. Steatocystoma. Dermatol Online J. 2013;19(12):17. PMID: 24365012.
  15. Marasca C, Megna M, Donnarumma M, Fontanella G, Cinelli E, Fabbrocini G. A case of steatocystoma multiplex in a psoriatic patient during treatment with Anti-IL-12/23. Skin Appendage Disord. 2020;6(5):309-311. doi: 10.1159/000507657.
  16. Atzori L, Zanniello R, Pilloni L, Rongioletti F. Steatocystoma multiplex suppurativa associated with hidradenitis suppurativa successfully treated with adalimumab. J Eur Acad Dermatol Venereol. 2019;33 (Suppl 6):42-44. doi: 10.1111/jdv.15848.
  17. Ha WW, Wang J, Wang W, et al. A novel missense mutation of keratin 17 gene in a Chinese family with steatocystoma multiplex. Ann Dermatol. 2013;25(4):508-510. doi: 10.5021/ad.2013.25.4.508.
  18. Kassira S, Korta DZ, de Feraudy S, Zachary CB. Fractionated ablative carbon dioxide laser treatment of steatocystoma multiplex. J Cosmet Laser Ther. 2016;18(7):364-366. doi: 10.1080/14764172.2016.1188212.
  19. Zussino M, Nazzaro G, Moltrasio C, Marzano AV. Coexistence of steatocystoma multiplex and hidradenitis suppurativa: Assessment of this unique association by means of ultrasonography and Color Doppler. Skin Res Technol. 2019;25(6):877-880. doi: 10.1111/srt.12751.
  20. Sharma A, Agrawal S, Dhurat R, Shukla D, Vishwanath T. An unusual case of facial steatocystoma multiplex: A clinicopathologic and dermoscopic report. Dermatopathology (Basel). 2018;5(2):58-63. doi: 10.1159/000488584.
  21. Waldemer-Streyer RJ, Jacobsen E. A tale of two cysts: Steatocystoma multiplex and eruptive vellus hair cysts-Two case reports and a review of the literature. Case Rep Dermatol Med. 2017;2017:3861972. doi: 10.1155/2017/3861972.
  22. Jeong SY, Kim JH, Seo SH, Son SW, Kim IH. Giant steatocystoma multiplex limited to the scalp. Clin Exp Dermatol. 2009;34(7):e318-319. doi: 10.1111/j.1365-2230.2009.03274.x.
  23. Mortazavi H, Taheri A, Mansoori P, Kani ZA. Localized forms of steatocystoma multiplex: case report and review of the literature. Dermatol Online J. 2005;11(1):22. PMID: 15748563.
  24. Lin KP, Chang ME, Ho WT. Treatment of steatocystoma multiplex on axillae using keyhole approach technique. J Cosmet Laser Ther. 2019;21(4):1. doi: 10.1080/14764172.2018.1525746.
  25. Kromann CB, Zarchi K, Nurnberg BM, Jemec GB. Recurring axillary, abdominal and genitofemoral nodules and abscesses: a quiz. Acta Derm Venereol. 2015;95(1):121-123. doi: 10.2340/00015555-1890.