Nevus Sebaceus Clinical Presentation
- Author: Anwar Al Hammadi, MD, FRCPC; Chief Editor: William D James, MD more...
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.
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. 
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 and pedunculated nodules has been reported.
The most common tumors arising within nevus sebaceous are syringocystadenoma papilliferum and trichoblastoma. A rare case of hybrid follicular cyst with matrical differentiation has been also reported.
Note the images below:
Nevus sebaceus lesions, especially when large, may be associated with multiple internal abnormalities, similar to those reported in linear epidermal nevus syndrome.
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.
Familial cases have been reported.[9, 10, 11] 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.
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. 2000 Apr. 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. 2005 Feb. 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. 2008 Sep. 22(9):1136-8. [Medline].
Baykal C, Buyukbabani N, Yazganoglu KD, Saglik E. [Tumors associated with nevus sebaceous]. J Dtsch Dermatol Ges. 2006 Jan. 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. 2000 Feb. 42(2 Pt 1):263-8. [Medline].
Correale D, Ringpfeil F, Rogers M. Large, papillomatous, pedunculated nevus sebaceus: a new phenotype. Pediatr Dermatol. 2008 May-Jun. 25(3):355-8. [Medline].
Rodins K, Baillie L. Hybrid follicular cyst (pilomatrical and infundibular) arising within a sebaceous nevus. Pediatr Dermatol. 2012 Mar-Apr. 29(2):213-6. [Medline].
Ivker R, Resnick SD, Skidmore RA. Hypophosphatemic vitamin D-resistant rickets, precocious puberty, and the epidermal nevus syndrome. Arch Dermatol. 1997 Dec. 133(12):1557-61. [Medline].
Fearfield LA, Bunker CB. Familial naevus sebaceous of Jadassohn. Br J Dermatol. 1998 Dec. 139(6):1119-20. [Medline].
Happle R, Konig A. Familial naevus sebaceus may be explained by paradominant transmission. Br J Dermatol. 1999 Aug. 141(2):377. [Medline].
Sahl WJ Jr. Familial nevus sebaceus of Jadassohn: occurrence in three generations. J Am Acad Dermatol. 1990 May. 22(5 Pt 1):853-4. [Medline].
Paller A, Mancini A, eds. Tumors of epidermal appendages. 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].
Idriss MH, Elston DM. Secondary neoplasms associated with nevus sebaceus of Jadassohn: a study of 707 cases. J Am Acad Dermatol. Feb2014. 70(2):332-7. [Medline].
Odom RB, James WD, Berger TG. Sebaceus nevi and tumors. Andrews' Diseases of the Skin. 9th ed. Philadelphia, Pa: WB Saunders; 2000. 845-46.