eMedicine Specialties > Dermatology > Benign Neoplasms

Nevus Sebaceus

Anwar Al Hammadi, MD, FRCPC, Consultant Dermatologist, Assistant Clinical Professor of Dermatology, University of Sharjah, Dubai, United Arab Emirates
Mark G Lebwohl, MD, Chairman, Department of Dermatology, Mount Sinai School of Medicine

Updated: Oct 23, 2009

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.

Differential Diagnoses

Aplasia Cutis Congenita

Other Problems to Be Considered

In early infancy, lesions in the scalp must be distinguished from cutis aplasia, which has a smoother papyraceous surface.

Nevus syringocystadenomatosus papilliferus may be difficult to distinguish at clinical examination, although the surface tends to be pink and nodular rather than yellow and velvety.

Early juvenile xanthogranulomas may be similar in their clinical appearance, although these generally develop rapidly into distinctive domed, papular, or nodular lesions.

Solitary mastocytomas may also be confused at clinical examination during infancy. Histologic examination clearly identifies all the disorders previously mentioned.

Congenital triangular alopecia should also be differentiated during infancy and childhood.

Workup

Histologic Findings

The epidermis shows papillomatous hyperplasia. In the dermis, the numbers of mature sebaceous glands are increased. Ectopic apocrine glands are often found in the deep dermis beneath sebaceous glands.

Frequently, small hair follicles and buds of basaloid cells that may represent malformed hair germs are present.

In childhood, the sebaceous glands in nevus sebaceus are underdeveloped, and the histologic findings may consist of only immature hair structures.

Mild papillomatosis of the epidermis with sebaceus gland lobules opening directly onto the epidermis.

Mild papillomatosis at high power.

Staging

In the early stage, the sebaceous glands and hair follicles are hypoplastic. In the second stage, at puberty, hyperkeratosis and papillomatosis with numerous and hyperplastic sebaceous glands (diagnostic) are present.

Treatment

Medical Care

Photodynamic therapy with topical aminolevulinic acid has been reported to have a good response for a nonsurgical ablative treatment in a limited number of cases.

Surgical Care

The risk of malignancy is difficult to establish with precision, and malignant change may occur at any age. Because of this risk, many authorities recommend complete surgical excision, preferably before puberty, because the lesion thickens and the risk of malignancy increases with age.
Full-thickness skin excision is usually required, and topical destruction is not recommended because it may mask malignant changes underneath the surface. Primary reconstruction is usually possible.

The timing of excision remains a matter of debate. Nevus sebaceus often is treated with observation until puberty because malignant degeneration is rare before this time. However, many factors need to be considered, including the size and the location of the nevus sebaceus, its cosmetic significance, and the risk-to-benefit ratio of general anesthesia, which is usually needed when the surgery is performed early in life, versus local anesthesia for surgery later in childhood or adolescence.¹¹

A 2007 study by Barkham et al concluded that prophylactic excision of all sebaceous nevi is not warranted, particularly in young children, and excision should be only recommended when benign or malignant neoplasms are clinically suspected or for cosmetic reasons.¹²

Carbon dioxide lasers have been used to treat a patient with involvement of the nose; however, the long-term risk of developing malignant transformation in any remaining deep dermal component must be considered.

Consultations

Patients should be examined for other associated findings as part of the linear nevus sebaceus syndrome. Pediatricians and other primary care providers working with the parents are usually the first to suspect or recognize neurologic or orthopedic abnormalities and to refer to the appropriate specialists.

• Consult a neurologist for epilepsy and other neurologic defects.
• Consult an orthopedist for skeletal deformities.

Follow-up

Complications

• Rapid, circumscribed enlargement, ulceration, or development of an exophytic nodule should raise suspicion of malignant transformation, although the development of benign appendageal tumors is considerably more common.
• The most common malignancy is basal cell carcinoma, but the incidence of this tumor has been overestimated because of misinterpretation of areas of basaloid proliferation as true basal cell carcinoma. Other malignant tumors reported include eccrine, squamous, sebaceous, and apocrine carcinomas.

Miscellaneous

Medicolegal Pitfalls

• Failure to recognize epidermal nevus syndrome
• Failure to closely monitor this congenital lesion for new changes, regardless of whether it was excised for prophylaxis (a less common procedure in current practice)

Multimedia

Media file 1: Nevus sebaceus in a 4-month-old baby manifesting as nodular plaque.

Media file 2: Brownish wartlike plaque in a 25-year-old patient.

Media file 3: Nevus sebaceus manifesting as a bald patch in a child.

Media file 4: Nevus sebaceus manifesting as an orange-yellow plaque with a smooth or somewhat velvety surface in a 6-month-old baby.

Media file 5: Nevus sebaceus manifesting as a small plaque beside a scaly scalp in a 13-year-old boy.

Media file 6: Linear type of nevus sebaceus.

Media file 7: Mild papillomatosis of the epidermis with sebaceus gland lobules opening directly onto the epidermis.

Media file 8: Mild papillomatosis at high power.

References

1. 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].

2. 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].

3. 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].

4. Baykal C, Buyukbabani N, Yazganoglu KD, Saglik E. [Tumors associated with nevus sebaceous]. J Dtsch Dermatol Ges. Jan 2006;4(1):28-31. [Medline].

5. 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].

6. Correale D, Ringpfeil F, Rogers M. Large, papillomatous, pedunculated nevus sebaceus: a new phenotype. Pediatr Dermatol. May-Jun 2008;25(3):355-8. [Medline].

7. 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].

8. Fearfield LA, Bunker CB. Familial naevus sebaceous of Jadassohn. Br J Dermatol. Dec 1998;139(6):1119-20. [Medline].

9. Happle R, Konig A. Familial naevus sebaceus may be explained by paradominant transmission. Br J Dermatol. Aug 1999;141(2):377. [Medline].

10. 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].

11. Paller A, Mancini A, eds. Tumors of epidermal appendages. In: Hurwitz Clinical Pediatric Dermatology. 3^rd ed. Philadelphia, Pa: Elsevier Saunders; 2006:221-2.

12. 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].

13. 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].

14. Elghamriny MS. Tumors with sebaceus gland differentiation. In: Ghamriny's Clinical Dermatology. Vol 2. 2000:658-59.

15. 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].

16. Happle R. Loss of heterozygosity in human skin. J Am Acad Dermatol. Aug 1999;41(2 Pt 1):143-64. [Medline].

17. Happle R. Mosaicism in human skin. Understanding the patterns and mechanisms. Arch Dermatol. Nov 1993;129(11):1460-70. [Medline].

18. 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.

19. 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].

20. McKee PH. Naevi and tumors of sebaceus gland derivation. In: Essential Skin Pathology. St. Louis: Mo: Mosby; 1999:169-70.

21. 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].

22. Odom RB, James WD, Berger TG. Sebaceus nevi and tumors. In: Andrews' Diseases of the Skin. 9^th ed. Philadelphia, Pa: WB Saunders; 2000:845-46.

Keywords

nevus sebaceus, sebaceous nevi, nevus sebaceus of Jadassohn, organoid nevus, verrucous epidermal nevi, epidermal nevus syndrome, Jadassohn nevus phakomatosis

Contributor Information and Disclosures

Author

Anwar Al Hammadi, MD, FRCPC, Consultant Dermatologist, Assistant Clinical Professor of Dermatology, University of Sharjah, Dubai, United Arab Emirates
Anwar Al Hammadi, MD, FRCPC is a member of the following medical societies: American Academy of Dermatology, Canadian Dermatology Association, Royal College of Physicians and Surgeons of Canada, and Skin Cancer Foundation
Disclosure: Nothing to disclose.

Coauthor(s)

Mark G Lebwohl, MD, Chairman, Department of Dermatology, Mount Sinai School of Medicine
Mark G Lebwohl, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Abbott Laboratories Honoraria Consulting; Actelion Honoraria Consulting; Amgen Honoraria Consulting; Astellas Honoraria Consulting; Centocor Honoraria Consulting; DermiPsor Honoraria Consulting; Galderma  Consulting; Genentech Honoraria Consulting; Helix BioMedix Honoraria Consulting; Medicis Honoraria Investigator

Medical Editor

Daniel Mark Siegel, MD, MS, Director, Procedural Dermatology Fellowship Program, Clinical Professor of Dermatology, Department of Dermatology, State University of New York Downstate
Daniel Mark Siegel, MD, MS is a member of the following medical societies: American Academy of Dermatology, American Academy of Facial Plastic and Reconstructive Surgery, American College of Mohs Micrographic Surgery and Cutaneous Oncology, American College of Physician Executives, American Society for Dermatologic Surgery, American Society for Laser Medicine and Surgery, American Society for MOHS Surgery, and International Society for Dermatologic Surgery
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Managing Editor

Van Perry, MD, Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas Health Science Center
Van Perry, MD is a member of the following medical societies: American Academy of Dermatology and American Society for Laser Medicine and Surgery
Disclosure: Nothing to disclose.

CME Editor

Catherine M Quirk, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania
Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

The authors and editors of eMedicine gratefully acknowledge the contributions of previous Chief Editor, William D. James, MD, to the development and writing of this article.

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