Nevus Sebaceus Clinical Presentation

  • Author: Anwar Al Hammadi, MD, FRCPC; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jun 9, 2010
 

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]

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Physical

Nevus sebaceus passes through 3 clinically distinct stages, as follows:

  • 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]

Note the images below:

Nevus sebaceus in a 4-month-old baby manifesting aNevus sebaceus in a 4-month-old baby manifesting as nodular plaque. Brownish wartlike plaque in a 25-year-old patient.Brownish wartlike plaque in a 25-year-old patient. Nevus sebaceus manifesting as a bald patch in a chNevus sebaceus manifesting as a bald patch in a child. Nevus sebaceus manifesting as an orange-yellow plaNevus 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 besidNevus sebaceus manifesting as a small plaque beside a scaly scalp in a 13-year-old boy. Linear type of nevus sebaceus. 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.

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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.

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Contributor Information and Disclosures
Author

Anwar Al Hammadi, MD, FRCPC  Consultant and Head of Dermatology, Rashid Hospital, Dubai Health Authority; Clinical Assistant Professor of Dermatology, University of Sharjah, 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; Amgen/Pfizer Honoraria Consulting; Astellas Honoraria Consulting; Centocor/Janssen Honoraria Consulting; DermiPsor Honoraria Consulting; GlaxoSmithKline Honoraria Consulting; Graceway Consulting; HelixBioMedix Honoraria Consulting; LEO Honoraria Consulting; Novartis Honoraria Consulting

Specialty Editor Board

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 College of Mohs Micrographic Surgery and Cutaneous Oncology, American College of Physician Executives, American Society for Dermatologic Surgery, American Society for MOHS Surgery, and International Society for Dermatologic Surgery

Disclosure: Nothing to disclose.

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.

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.

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.

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. 3rd 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. 9th ed. Philadelphia, Pa: WB Saunders; 2000:845-46.

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