Baylor University Medical Center Proceedings April 2017 - 212

A sebaceous nevus, or nevus sebaceous of Jadassohn, is a benign lesion classiﬁed as a hamartoma consisting of sebaceous and
apocrine glands and epidermal and follicular elements (3). These
lesions are most often present at birth, classically appearing on
the scalp as an alopecic area of a yellow-orange, linear, crescentic,
or round plaque with a waxy sheen (4-6). Histologically, biopsy
may reveal immature hair follicles, ectopically located apocrine
glands, and abundant sebaceous glands sitting uncharacteristically superﬁcial in the dermis (7). Sebaceous nevi often enlarge
near the time of puberty due to sebaceous gland hyperplasia,
apocrine gland maturation, and epidermal hyperplasia (8). The
verrucous appearance that often develops at this time may incite
initial presentation to dermatology or primary care.
Initial workup of these lesions should include a clinical
evaluation for nevus sebaceous syndrome, or Schimmelpenning
syndrome. Nevus sebaceous syndrome is characterized by diﬀuse
sebaceous nevi on the face or scalp and is associated with central
nervous system abnormalities including seizures and mental
retardation, ocular impairments, and skeletal defects such as
craniofacial abnormalities and hypophosphatemic rickets resistant to vitamin D (9-11). Thus, in pediatric patients especially,
the presence of a sebaceous nevus should be documented with a
brief neurologic, ocular, and musculoskeletal exam. Any patient
suspected of having nevus sebaceous syndrome should undergo
a more thorough physical exam with possible laboratory and/
or imaging evaluations.
The most frequent complications of a sebaceous nevus
are increased size, nodularity, and alopecia, which may result
in undesirable cosmesis (6). Serious complications can occur
such as the development of a secondary neoplasm and a risk of
malignant transformation (6, 12, 13). The rate of malignancy
remains unknown due a lack of prospective studies, but current
literature suggests that the most common secondary neoplasms
seen within sebaceous nevi are benign syringocystadenoma papilliferum and trichoblastomas (6, 13-16). There is a known association with basal cell carcinomas, which have been reported
to occur in <1% of patients with sebaceous nevi (13-15). Many
other malignant tumors have been reported to be associated
with sebaceous nevi, including squamous cell carcinomas, sebaceous carcinomas, and apocrine carcinomas (6, 13, 17). Despite
the overall risk of malignancy increasing with age, Rosen et al
showed that basal cell carcinomas can develop within these
lesions even prior to puberty and without signiﬁcant physical change (14). Conversely, Santibanez-Gallerani et al studied
757 cases of sebaceous nevi in children <16 years of age and
found no basal cell carcinomas, thereby drawing the conclusion
that prophylactic excision is not indicated (16). Determining
workup and management of these lesions requires acknowledgment of these risks and consideration of patient goals.
Treatment of a sebaceous nevus is controversial. Historically,
it was believed that prophylactic excision was necessary in all

212

cases due to the risk of malignant transformation, but newer
studies have shown that the rate of malignancy was grossly
overestimated and have drawn conﬂicting conclusions. Some
authors have concluded that observation only is acceptable and
excisional biopsy is not necessary, while others still propose
prophylactic excision, with disagreements on the timing of treatment (5, 13-15). If treatment is desired, deﬁnitive removal of
the lesion requires full-thickness surgical excision (14). This may
be preferred by some patients or parents for cosmetic outcomes
or may be indicated due to clinical signs of malignancy such
as rapid growth, ulceration, or bleeding (12). Treatment timing must consider the age of the patient, the need for general
versus local anesthesia, the size of the lesion, and overall goals of
treatment (14). Due to the inconsistency of treatment recommendations in the literature, management of a sebaceous nevus
must be decided upon by the clinician and the patient with
consideration of the associated risks accompanying each option.
1.
2.
3.
4.
5.

6.
7.

8.
9.
10.
11.
12.
13.

14.

15.
16.

17.

Thiboutot D. Regulation of human sebaceous glands. J Invest Dermatol
2004;123(1):1-12.
Plewig G, Kligman AM. Proliferative activity of the sebaceous glands of
the aged. J Invest Dermatol 1978;70(6):314-317.
Jadassohn J. Bemerkugen zur Histologie der systematisirten Naevi and
ueber "Talgdruesen-navi." Arch Derm Syphilol 1895;33(1):355-394.
Conner AE, Bryan H. Nevus sebaceous of Jadassohn. Am J Dis Child
1967;114(6):626-630.
Jaqueti G, Requena L, Sánchez 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;22(2):108-118.
Brandling-Bennett HA, Morel KD. Epidermal nevi. Pediatr Clin North
Am 2010;57(5):1177-1198.
Simi CM, Rajalakshmi T, Correa M. Clinicopathologic analysis of 21 cases
of nevus sebaceus: a retrospective study. Indian J Dermatol Venereol Leprol
2008;74(6):625-627.
Mehregan AH, Pinkus H. Life history of organoid nevi. Special reference
to nevus sebaceus of Jadassohn. Arch Dermatol 1965;91(6):574-588.
Happle R, Konig A. Familial naevus sebaceus may be explained by
paradominant transmission. Br J Dermatol 1999;141(2):377.
Sugarman JL. Epidermal nevus syndromes. Semin Cutan Med Surg
2007;26(4):221-230.
Davies D, Rogers M. Review of neurological manifestations in 196
patients with sebaceous naevi. Australas J Dermatol 2002;43(1):20-23.
Eisen DB, Michael DJ. Sebaceous lesions and their associated syndromes:
part I. J Am Acad Dermatol 2009;61(4):549-560.
Idriss MH, Elston DM. Secondary neoplasms associated with nevus
sebaceus of Jadassohn: a study of 707 cases. J Am Acad Dermatol
2014;70(2):332-337.
Rosen H, Schmidt B, Lam HP, Meara JG, Labow BI. Management of
nevus sebaceous and the risk of basal cell carcinoma: an 18-year review.
Pediatr Dermatol 2009;26(6):676-681.
Cribier B, Scrivener Y, Grosshans E. Tumors arising in nevus sebaceus: a
study of 596 cases. J Am Acad Dermatol 2000;42(2 Pt 1):263-268.
Santibanez-Gallerani A, Marshall D, Duarte AM, Melnick SJ, Thaller S.
Should nevus sebaceus of Jadassohn in children be excised? A study of
757 cases, and literature review. J Craniofac Surg 2003;14(5):658-660.
Domingo J, Helwig EB. Malignant neoplasms associated with nevus
sebaceus of Jadassohn. J Am Acad Dermatol 1979;1(6):545-556.

Baylor University Medical Center Proceedings

Volume 30, Number 2

Table of Contents for the Digital Edition of Baylor University Medical Center Proceedings April 2017