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Dermatology > BENIGN NEOPLASMS
Nevus Sebaceus
Article Last Updated: Nov 12, 2008
AUTHOR AND EDITOR INFORMATION
Section 1 of 10  
Author: Anwar Al Hammadi, MD, FRCPC, Consultant Dermatologist, Assistant Clinical Professor of Dermatology, University of Sharjah, Dubai, United Arab Emirates
Anwar Al Hammadi 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
Coauthor(s):
Mark G Lebwohl, MD, Chairman, Department of Dermatology, Mount Sinai School of Medicine
Editors: Daniel Mark Siegel, MD, MS, Director, Procedural Dermatology Fellowship Program, Clinical Professor of Dermatology, Department of Dermatology, State University of New York Downstate; 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; Van Perry, MD, Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas Health Science Center; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
Author and Editor Disclosure
Synonyms and related keywords:
nevus sebaceus of Jadassohn, organoid nevus, verrucous epidermal nevi, epidermal nevus syndrome, Jadassohn nevus phakomatosis
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
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.
International
Lesions are sporadic and occur with equal frequency in males and females of all races.
Mortality/Morbidity
The medical importance of a solitary nevus sebaceous 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 recent series suggest that this rate may be much lower. These neoplasms 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. Recent 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.
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.
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.
- 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.
- Nevus sebaceus lesions, especially when large, may be associated with multiple internal abnormalities, similar to those reported in linear epidermal nevus syndrome.3
- 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.
- Recently, a case of linear squamous cell papilloma associated with sebaceus nevus syndrome was described in a 7-year-old boy.
Causes
- Familial cases have been reported.4, 5, 6 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 sebaceous and may be responsible for the predisposition to the development of basal cell carcinoma and other tumors in this lesion.
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.
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 sebaceous are underdeveloped, and the histologic findings may consist of only immature hair structures.
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.
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. These lesions often are treated with observation until puberty because malignant degeneration is rare before this time. However, early removal is preferred because the patient may avoid social consequences that a prominent lesion may invoke from peers during the formative years. A 2007 study by Barkham et al7 concluded that prophylactic excision of all sebaceus 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. 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.
The Medscape Dermatologic Surgery Resource Center may be helpful.
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.
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.
Medical/Legal 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)
| Media file 1:
Brownish wartlike plaque in a 25-year-old patient. |
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| Media file 2:
Nevus sebaceus manifesting as a bald patch in a child. |
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| Media file 3:
Nevus sebaceus manifesting as an orange-yellow plaque with a smooth or somewhat velvety surface in a 6-month-old baby. |
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| Media file 4:
Nevus sebaceus manifesting as a small plaque beside a scaly scalp in a 13-year-old boy. |
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| Media file 6:
Mild papillomatosis of the epidermis with sebaceus gland lobules opening directly onto the epidermis. |
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- Warnke PH, Russo PA, Schimmelpenning GW, Happle R, Härle F, Hauschild A, et al. Linear intraoral lesions in the sebaceous nevus syndrome. J Am Acad Dermatol. Feb 2005;52(2 Suppl 1):62-4. [Medline].
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Nevus Sebaceus excerpt Article Last Updated: Nov 12, 2008
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