Introduction
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.
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.
Clinical
History
- Most frequently, a solitary, hairless patch is noted on the scalp at birth or in early childhood. A velvety tan or orange-yellow plaque may also occur on other areas of the head and the neck.
- Hormonal influences from the mother may briefly increase the prominence in an infant, whereas pubertal hormones enhance the verrucoid appearance in an adolescent.
- Nevus sebaceus has a predilection for the scalp (vertex) and less commonly occurs on the face, around the ears, on the neck, or on the trunk. Nevus sebaceus occurring exclusively in the oral cavity has also been reported.2
Physical
- Nevus sebaceus passes through 3 clinically distinct stages.
- At birth or in early infancy, nevus sebaceus appears as a hairless, solitary, linear or round, slightly raised, pinkish, yellow, orange, or tan plaque, with a smooth or somewhat velvety surface. The nevus is usually on the scalp, often near the vertex or on the face. Extensive lesions not limited to the head have been reported.3
- In adolescence, the lesion becomes verrucous and nodular, round, oval, or linear in shape, varying in length from about 1 cm to more than 10 cm. They most commonly occur as a single lesion, but they may be multiple and extensive.
- Later in life, some lesions may develop various types of appendageal tumors, such as trichoblastoma; syringocystadenoma papilliferum; basal cell carcinoma; and, less commonly, nodular hidradenoma, sebaceous epithelioma, apocrine cystadenoma, eccrine carcinoma, squamous cell carcinoma, sebaceous carcinoma, spiradenoma, and keratoacanthoma.4,5
- An unusual phenotype with large, pink, exophytic nodules has been reported.6
Nevus sebaceus in a 4-month-old baby manifesting as nodular plaque.
Brownish wartlike plaque in a 25-year-old patient.
Nevus sebaceus manifesting as a bald patch in a child.
Nevus sebaceus manifesting as an orange-yellow plaque with a smooth or somewhat velvety surface in a 6-month-old baby.
Nevus sebaceus manifesting as a small plaque beside a scaly scalp in a 13-year-old boy.
Linear type of nevus sebaceus.
- Nevus sebaceus lesions, especially when large, may be associated with multiple internal abnormalities, similar to those reported in linear epidermal nevus syndrome.7
- Associated problems may include intracranial masses, seizures, mental retardation, skeletal abnormalities, pigmentary changes, ocular lesions, and hamartomas of the kidney. Mediastinal lipomatosis has also been reported.
- Epidermal nevus syndrome (Jadassohn nevus phakomatosis) is the combination of extensive sebaceous nevi with disorders of the central nervous system, the bone, and the eye. Some of the more common abnormalities include epilepsy; mental retardation; seizures or other neurologic defects; skeletal deformities, such as vitamin D–resistant rickets, spina bifida, bone hyperplasia, or bone hypertrophy; and ocular lesions, such as ptosis, nystagmus, optic nerve hypoplasia, and oculomotor dysfunction.
- A case of linear squamous cell papilloma associated with sebaceous nevus syndrome has been described in a 7-year-old boy.
Causes
- Familial cases have been reported.8,9,10 Mutations in pluripotential cells during embryogenesis may generate varying lines of differentiation included in organoid nevi. Nevus sebaceus appears to respond to hormonal influences, as the lesion can be raised at birth, become flattened in childhood, and become raised again during puberty.
- Deletions of the patched gene have been identified in nevus sebaceus and may be responsible for the predisposition to the development of basal cell carcinoma and other tumors in this lesion.
More on Nevus Sebaceus |
 Overview: Nevus Sebaceus |
| Differential Diagnoses & Workup: Nevus Sebaceus |
| Treatment & Medication: Nevus Sebaceus |
| Follow-up: Nevus Sebaceus |
| Multimedia: Nevus Sebaceus |
| References |
| Next Page » |
References
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.
Further Reading
Keywords
nevus sebaceus, sebaceous nevi, nevus sebaceus of Jadassohn, organoid nevus, verrucous epidermal nevi, epidermal nevus syndrome, Jadassohn nevus phakomatosis
