Nevus sebaceus (sebaceous nevus), a circumscribed hamartomatous lesion predominantly composed of sebaceous glands, was first described by Jadassohn in 1895. It is a rare benign tumor in children that usually presents with warty patches of hair loss on the scalp (see the image below). Sebaceous nevi and verrucous epidermal nevi are closely related, and many authors regard them as variants.
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Nevus sebaceus manifesting as a bald patch in a child.
Nevus sebaceus is a rare benign tumor in children that usually presents with warty patches of hair loss on the scalp.
The development of secondary malignant neoplasms within the nevus sebaceus is rare and occurs almost exclusively in adults. Possible signs of malignancy include ulceration or a new "bump" on the area; thus if any change is seen within the nevus sebaceus, patients should seek medical advice. Given the low risk of malignant transformation in children, clinical follow-up is considered to be a safe alternative to prophylactic surgical excision in pediatric patients.
If treatment is chosen, surgical excision would be the treatment of choice[1] ; however, the timing of surgery is controversial.[2] Factors to be considered include the size and location of the nevus, its cosmetic significance, and the risks and benefits of early excision (which usually requires general anesthesia) vs delayed excision (which usually requires only local anesthesia). Laser ablation has been employed for treatment as well.[3]
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.
Familial cases of sebaceous nevus 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; 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 (BCC) and other tumors in this lesion.
In the United States, nevus sebaceus occurs with equal frequency in males and females of all races; it affects 0.3% of newborns. Worldwide, sebaceous nevi are sporadic and occur with equal frequency in males and females of all races.
Nevus sebaceus is usually noted as a solitary lesion at birth or in early childhood, whereas the characteristic features may not develop until puberty.
The medical importance of a solitary nevus sebaceus relates to the description of both benign change and, in some cases, malignant neoplastic change. Whereas earlier studies reported malignant transformation rates in the range of 10-15%, subsequent studies demonstrated that the rate is certainly less than 1% and that transformation nearly always occurs after puberty. In older reports, many lesions misdiagnosed as BCCs were in fact trichoblastomas (benign neoplasms that resemble BCC histologically).
The most common malignant neoplasm arising in this disorder is BCC, but the development of this or any other malignant neoplasm is uncommon. The most frequent benign tumors are trichoblastomas and syringocystadenoma papilliferum, occurring in fewer than 5% of patients with nevus sebaceus.[7]
Other benign and malignant tumors include apocrine cystadenoma, leiomyoma and sebaceous cell carcinoma. Rarely, malignant eccrine poromas, sebaceous carcinomas, and apocrine carcinomas have been reported to result in widespread metastases and death.
Most frequently, a solitary hairless patch is noted on the scalp at birth or in early childhood (see the image below). A velvety tan or orange-yellow plaque may also occur on other areas of the head and the neck.
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Nevus sebaceus manifesting as a bald patch in a child.
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 (sebaceous nevus) 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.[8]
Nevus sebaceus passes through three clinically distinct stages, as follows (see the images below):
Stage 1 - 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[9]
Stage 2 - In adolescence, the lesion becomes verrucous and nodular, round, oval, or linear in shape, ranging in length from about 1 cm to more than 10 cm; the nevi most commonly occur as a single lesion, but they may be multiple and extensive
Stage 3 - Later in life, some lesions may develop various types of appendageal tumors, such as trichoblastoma; syringocystadenoma papilliferum; basal cell carcinoma (BCC); and, less commonly, nodular hidradenoma, sebaceous epithelioma, apocrine cystadenoma, eccrine carcinoma, squamous cell carcinoma (SCC), sebaceous carcinoma, spiradenoma, and keratoacanthoma[10, 11, 12]
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Nevus sebaceus in a 4-month-old baby manifesting as nodular plaque.
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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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Nevus sebaceus manifesting as a small plaque beside a scaly scalp in a 13-year-old boy.
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Linear type of nevus sebaceus.
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Verrucous plaque in a 19-year-old woman.
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Brownish wartlike plaque in a 25-year-old patient.
An unusual phenotype with large, pink, exophytic and pedunculated nodules has been reported.[13]
The most common tumors arising within nevus sebaceus are syringocystadenoma papilliferum and trichoblastoma. Rarely, hybrid follicular cyst with matrical differentiation has been reported.[14]
Nevus sebaceus lesions, especially when large, may be associated with multiple internal abnormalities, similar to those reported in linear epidermal nevus syndrome.[15]
Associated problems may include intracranial masses, seizures, intellectual disability, skeletal abnormalities, pigmentary changes, ocular lesions, and hamartomas of the kidney. Mediastinal lipomatosis has also been reported.
Schimmelpenning syndrome is the combination of extensive sebaceous nevi with disorders of the central nervous system (CNS), the skeleton, and the eyes. Among the more common abnormalities are epilepsy; intellectual disability; 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.
Rapid, circumscribed enlargement, ulceration, or development of an exophytic nodule should raise suspicion of malignant transformation, though the development of benign appendageal tumors is considerably more common.
The most common malignancy is BCC, but the incidence of this tumor has been overestimated in the past when areas of basaloid proliferation were misinterpreted as representing true BCC. Other malignant tumors reported include eccrine, squamous, sebaceous, and apocrine carcinomas.
The epidermis shows papillomatous hyperplasia. (See the images below.) In the dermis, the numbers of mature sebaceous glands are increased. Ectopic apocrine glands are often found in the deep dermis beneath sebaceous glands.
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Mild papillomatosis of the epidermis with sebaceus gland lobules opening directly onto the epidermis.
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Mild papillomatosis at high power.
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.
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.
Photodynamic therapy with topical aminolevulinic acid has been reported to have a good response as a nonsurgical ablative treatment for nevus sebaceus (sebaceous nevus) in a limited number of cases.[16] A case series suggested that topical sirolimus might prove useful in the treatment of nevus sebaceus, particularly in patients who decline surgical treatment.[17]
The risk of malignancy is low, and if malignant transformation does occur, it is almost always after puberty. Unless stigmatization, disfigurement, symptomatology, or a growing tumor within the lesion is present, excision is typically delayed until adulthood, when the patient may participate in the decision.[18]
Full-thickness skin excision is usually required; topical destruction generally is not recommended because it may mask malignant changes underneath the surface. Primary reconstruction is usually possible.
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 recommended only when benign or malignant neoplasms are clinically suspected or for cosmetic reasons.[19]
A 2014 retrospective analysis of 707 cases of nevus sebaceus diagnosed at the Ackerman Academy of Dermatopathology from 1999 to 2012 confirmed that most of the secondary neoplasms arising in association with nevus sebaceus are benign. Because no malignant tumors were seen in children, the authors concluded that it is reasonable to delay surgical management until adolescence.[20]
Lasers have been used in the treatment of nevus sebaceus; however, the long-term risk of developing malignant transformation in any remaining deep dermal component must be considered. A 2022 S2k guideline from Germany recommended the use of either a 10,600-nm carbon dioxide laser or a 2940-nm Er:YAG laser to treat nevus sebaceus.[3]
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.
What is nevus sebaceus?What is the pathophysiology of nevus sebaceus?What causes nevus sebaceus?What is the prevalence of nevus sebaceus in the US?What are racial predilections of nevus sebaceus?What is the global prevalence of nevus sebaceus?What are the sexual predilections of nevus sebaceus?At what ages does nevus sebaceus typically present?What is the prognosis of nevus sebaceus?What is included in patient education about nevus sebaceus?Which clinical history findings are characteristic of nevus sebaceus?What are the clinical stages of nevus sebaceus?Which physical findings are characteristic of nevus sebaceus?What are the possible complications of nevus sebaceus?Which conditions should be included in the differential diagnoses of nevus sebaceus?What are the differential diagnoses for Nevus Sebaceus?Which histologic findings are characteristic of nevus sebaceus?What are the stages of nevus sebaceus?What is the role of photodynamic therapy in the treatment of nevus sebaceus?What is the role of surgery in the treatment of nevus sebaceus?Which specialist consultations are beneficial for patients with nevus sebaceus?
Anwar Al Hammadi, MD, FRCPC, Consultant and Head of Dermatology, Rashid Hospital, Dubai Health Authority; Clinical Associate Professor of Dermatology, Dubai Medical College; Clinical Assistant Professor of Dermatology, University of Sharjah, UAE
Disclosure: Nothing to disclose.
Coauthor(s)
Mark G Lebwohl, MD, Chairman, Department of Dermatology, Mount Sinai School of Medicine
Disclosure: Received none from Amgen for consultant & investigator; Received none from Novartis for consultant & investigator; Received none from Pfizer for consultant & investigator; Received none from Celgene Corporation for consultant & investigator; Received none from Clinuvel for consultant & investigator; Received none from Eli Lilly & Co. for consultant & investigator; Received none from Janssen Ortho Biotech for consultant & investigator; Received none from LEO Pharmaceuticals for consultant & inves.
Specialty Editors
David F Butler, MD, Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic
Disclosure: Nothing to disclose.
Van Perry, MD, Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas School of Medicine at San Antonio
Disclosure: Nothing to disclose.
Chief Editor
William D James, MD, Emeritus Professor, Department of Dermatology, University of Pennsylvania School of Medicine
Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.
Additional Contributors
Daniel Mark Siegel, MD, MS, Clinical Professor of Dermatology, Department of Dermatology, State University of New York Downstate Medical Center
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