STEATOCYSTOMA MULTIPLEX IN AN ADOLESCENT BOY: A CASE REPORT

Authors

  • Tomica Sotirovski University Clinic for Dermatology, Faculty of Medicine, Ss. Cyril and Methodius University, Skopje, Republic of North Macedonia
  • Ivana Tusheva University Clinic for Dermatology, Faculty of Medicine, Ss. Cyril and Methodius University, Skopje, Republic of North Macedonia
  • Ana Blazeska University Clinic for Dermatology, Faculty of Medicine, Ss. Cyril and Methodius University, Skopje, Republic of North Macedonia
  • Marija Djambazova University Clinic for Dermatology, Faculty of Medicine, Ss. Cyril and Methodius University, Skopje, Republic of North Macedonia
  • Igor Peev University Clinic for Plastic and Reconstructive Surgery, Ss. Cyril and Methodius University in Skopje, Faculty of Medicine in Skopje, Republic of North Macedonia
  • Katerina Damevska University Clinic for Dermatology, Faculty of Medicine, Ss. Cyril and Methodius University, Skopje, Republic of North Macedonia

Keywords:

Steatocystoma multiplex, Keratin-17, hereditary, cyst, acne, genodermatosis

Abstract

Steatocystoma multiplex (SM) is a rare genodermatosis transmitted as an autosomal dominant trait caused most often by a mutation in the gene coding for keratin 17. There are some sporadic cases described in the literature. The disease commonly manifests as numerous intradermal cysts caused by hamartomatous malformations of the pilosebaceous duct junction. The cysts can be located in any skin region, mainly on the chest. The disease is usually benign and asymptomatic, but it can be exceptionally disfiguring, which is the main reason for a medical visit. Pachyonychia congenita type-2 and the eruptive vellus hair cyst are closely related to SM; therefore, histopathological confirmation is necessary before starting any treatment. Here, we present a case of a widespread SM in an adolescent boy, focusing on its clinical and histopathological characteristics.

References

Shin NY, Kang JH, Kim JE, Symkhampa K, Huh KH, Yi WJ, et al. Steatocystoma multiplex: A case report of a rare entity. Imaging Sci Dent 2019; 49(4): 317-321. doi: 10. 5624/isd.2019.49.4.317.

Amin M, Hashim P. Steatocystoma Multiplex: Case Report and Review of Treatment. SKIN The Journal of Cutaneous Medicine 2018; 2(1): 75-79. doi.org/10.25251/ skin.2.1.12.

Georgakopoulos JR, Ighani A, Yeung J. Numerous asymptomatic dermal cysts: diagnosis and treatment of steatocystoma multiplex. Can Fam Physician 2018; 64(12): 892-899. PMID: 30541803; PMCID: PMC6371868.

Kamra HT, Gadgil PA, Ovhal AG, Narkhede RR. Steatocystoma multiplex-a rare genetic disorder: a case report and review of the literature. J Clin Diagn Res 2013; 7(1): 166-168. doi: 10.7860/JCDR/2012/4691.2698.

Chu HD. Steatocystoma multiplex. Dermatology Online J. 2013; 9(4):18. 2013 doi: 10.7860/JCDR/2012/4691.2698.

Wang X, Shi Y, Ye Y, Liu F, Jin W, Chen W, et al. Keratin 17 gene mutation in patients with steatocystoma multiplex. Zhonghua Yi Xue Za Zhi 2001; 81(9): 540-543. PMID: 11809119.

Choudhary S, Koley S, Salodkar A. A modified surgical technique for steatocystoma multiplex. J Cutan Aesthet Surg 2010; 3(1): 25-28. doi: 10.4103/0974-2077.63284.

Kamra HT, Gadgil PA, Ovhal AG, Narkhede RR. Steatocystoma multiplex-a rare genetic disorder: a case report and review of the literature. J Clin Diagn Res 2013; 7(1): 166-168. doi: 10.7860/JCDR/2012/4691.2698.

Gass JK, Wilson NJ, Smith FJ, Lane EB, McLean WH, Rytina E, et al. Steatocystoma multiplex, oligodontia and partial persistent primary dentition associated with a novel keratin 17 mutation. Br J Dermatol 2009; 161(6): 1396-1398. doi: 10.1111/j.1365-2133. 2009.09383.x.

Antal AS, Kulichova D, Redler S, Betz RC, Ruzicka T. Steatocystoma multiplex: keratin 17 - the key player? Br J Dermatol 2012; 167(6): 1395-1397. doi: 10.1111/j.1365-2133.2012.11073.x.

Pamoukian VN, Westreich M. Five generations with steatocystoma multiplex congenita: a treatment regimen. Plast Reconstr Surg 1997; 99(4): 1142-1146. doi: 10.1097/00006534-199704000-00036.

Kim SJ, Park HJ, Oh ST, Lee JY, Cho BK. A case of steatocystoma multiplex limited to the scalp. Ann Dermatol 2009; 21(1): 106-109. doi: 10.5021/ad.2009.21.1.106.

Covello SP, Smith FJ, Sillevis Smitt JH, Paller AS, Munro CS, Jonkman MF, et al. Keratin 17 mutations cause either steatocystoma multiplex or pachyonychia congenita type 2. Br J Dermatol 1998; 139(3): 475-480. doi: 10.1046/j.1365-2133.1998.02413.x.

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Published

2023-03-31

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Section

Case Reports