Background
In 1895, Jadassohn first described nevus sebaceus, a circumscribed hamartomatous lesion predominantly composed of sebaceous glands. Sebaceous nevi and verrucous epidermal nevi are closely related, and many authors regard them as variants.
Pathophysiology
In nevus sebaceus, postzygotic somatic mutations may result in various clinical expressions of mosaicism. Mutations in pluripotential cells may give rise to hamartomas with multiple cell lines.
Epidemiology
Frequency
United States
Nevus sebaceus occurs with equal frequency in males and females of all races. Of newborns, 0.3% are affected by nevus sebaceus.
International
Sebaceous nevi are sporadic and occur with equal frequency in males and females of all races.
Mortality/Morbidity
The medical importance of a solitary nevus sebaceus relates to the description of both benign change and, in some cases, malignant neoplastic change. Malignant transformation occurs in 10-15% of lesions in some series, although series suggest that this rate may be much lower. Sebaceous nevi generally occur during adolescence or adult life. Rarely, changes have occurred in children younger than 5 years. The most common malignant neoplasm arising in this disorder is basal cell carcinoma. Studies indicate that the development of basal cell carcinoma or any other malignant neoplasm is very rare. The most frequent benign tumor is trichoblastoma.[1]
Other benign and malignant tumors include syringocystadenoma papilliferum arising from the apocrine sweat glands, keratoacanthoma, apocrine cystadenoma, leiomyoma, and sebaceous cell carcinoma. Rarely, malignant eccrine poromas and apocrine carcinomas have been reported to result in widespread metastases and death.
Race
Nevus sebaceus occurs with equal frequency in males and females of all races.
Sex
Males and females are equally affected by nevus sebaceus.
Age
Nevus sebaceus is usually noted as a solitary lesion at birth or in early childhood, whereas the characteristic features may not develop until puberty.
Jaqueti G, Requena L, Sanchez Yus E. Trichoblastoma is the most common neoplasm developed in nevus sebaceus of Jadassohn: a clinicopathologic study of a series of 155 cases. Am J Dermatopathol. Apr 2000;22(2):108-18. [Medline].
Warnke PH, Russo PA, Schimmelpenning GW, et al. Linear intraoral lesions in the sebaceous nevus syndrome. J Am Acad Dermatol. Feb 2005;52(2 Suppl 1):62-4. [Medline].
Kavak A, Ozcelik D, Belenli O, Buyukbabani N, Saglam I, Lazova R. A unique location of naevus sebaceus: labia minora. J Eur Acad Dermatol Venereol. Sep 2008;22(9):1136-8. [Medline].
Baykal C, Buyukbabani N, Yazganoglu KD, Saglik E. [Tumors associated with nevus sebaceous]. J Dtsch Dermatol Ges. Jan 2006;4(1):28-31. [Medline].
Cribier B, Scrivener Y, Grosshans E. Tumors arising in nevus sebaceus: A study of 596 cases. J Am Acad Dermatol. Feb 2000;42(2 Pt 1):263-8. [Medline].
Correale D, Ringpfeil F, Rogers M. Large, papillomatous, pedunculated nevus sebaceus: a new phenotype. Pediatr Dermatol. May-Jun 2008;25(3):355-8. [Medline].
Ivker R, Resnick SD, Skidmore RA. Hypophosphatemic vitamin D-resistant rickets, precocious puberty, and the epidermal nevus syndrome. Arch Dermatol. Dec 1997;133(12):1557-61. [Medline].
Fearfield LA, Bunker CB. Familial naevus sebaceous of Jadassohn. Br J Dermatol. Dec 1998;139(6):1119-20. [Medline].
Happle R, Konig A. Familial naevus sebaceus may be explained by paradominant transmission. Br J Dermatol. Aug 1999;141(2):377. [Medline].
Sahl WJ Jr. Familial nevus sebaceus of Jadassohn: occurrence in three generations. J Am Acad Dermatol. May 1990;22(5 Pt 1):853-4. [Medline].
Paller A, Mancini A, eds. Tumors of epidermal appendages. In: Hurwitz Clinical Pediatric Dermatology. 3rd ed. Philadelphia, Pa: Elsevier Saunders; 2006:221-2.
Barkham MC, White N, Brundler MA, Richard B, Moss C. Should naevus sebaceus be excised prophylactically? A clinical audit. J Plast Reconstr Aesthet Surg. 2007;60(11):1269-70. [Medline].
Beer GM, Widder W, Cierpka KA, Kompatscher P, Meyer VE. [The development of malignant tumors in nevus sebaceus--therapeutic consequences]. Wien Klin Wochenschr. Mar 26 1999;111(6):236-9. [Medline].
Elghamriny MS. Tumors with sebaceus gland differentiation. In: Ghamriny's Clinical Dermatology. Vol 2. 2000:658-59.
Hamilton KS, Johnson S, Smoller BR. The role of androgen receptors in the clinical course of nevus sebaceus of Jadassohn. Mod Pathol. Jun 2001;14(6):539-42. [Medline].
Happle R. Loss of heterozygosity in human skin. J Am Acad Dermatol. Aug 1999;41(2 Pt 1):143-64. [Medline].
Happle R. Mosaicism in human skin. Understanding the patterns and mechanisms. Arch Dermatol. Nov 1993;129(11):1460-70. [Medline].
Ho VC, Freedberg IM, Eisen AZ, Wolff K. Benign epithelial tumors (nevus sebaceus). In: Fitzpatrick's Dermatology In General Medicine. Vol 1. New York, NY: McGraw-Hill; 1999:878-79.
Lupton JR, Elgart ML, Sulica VI. Segmental neurofibromatosis in association with nevus sebaceus of Jadassohn. J Am Acad Dermatol. Nov 2000;43(5 Pt 2):895-7. [Medline].
McKee PH. Naevi and tumors of sebaceus gland derivation. In: Essential Skin Pathology. St. Louis: Mo: Mosby; 1999:169-70.
Moody BR, Hurt MA. Surgical management of the cutaneous manifestations of linear nevus sebaceus syndrome. Plast Reconstr Surg. Feb 2004;113(2):799-800. [Medline].
Odom RB, James WD, Berger TG. Sebaceus nevi and tumors. In: Andrews' Diseases of the Skin. 9th ed. Philadelphia, Pa: WB Saunders; 2000:845-46.

