Evaluation and management of an unusual congenital nevus
K. Blaire Kerwin, BS, and M. Alan Menter, MD
Abnormal findings on routine skin exams are common and can be a
source of unnecessary medical workup if a clinician is unfamiliar with the
finding. Sebaceous nevi are rare skin lesions that are most often benign
but may be associated with a multiorgan syndrome or local skin cancer.
Dermatologists and primary care physicians may encounter these on
routine exams and thus must be comfortable with diagnosis and management. We present the clinical characteristics of a benign sebaceous
nevus to help aid in diagnosis of these lesions and outline suggestions
for appropriate management options.
T
he total body skin examination is one of the most common dermatologic visits and a common component of
primary care visits. Examining the skin for potential
malignancies can be taxing; therefore, efficient evaluation is a necessity for high-volume clinics. Quick identification of malignant features and confidence in evaluating rare
nevi is fundamental to efficiency. We present a case describing
the evaluation and management of a rare, unusual-appearing
congenital nevus with neoplastic potential.
CASE DESCRIPTION
A 52-year-old white woman presented to the dermatology
clinic for a routine annual full-body skin exam and evaluation
of a suspicious lesion she noticed on her posterior auricular
skinfold. She was unsure how long the lesion had been present
but believed it to be growing in size over the past 5 years. She
denied pain, pruritus, bleeding, or drainage from the lesion
and denied ophthalmologic defects, neurologic symptoms, skeletal deformities, or a personal history of skin cancers.
The postauricular lesion was a linear, waxy, flesh- and yellowcolored plaque that measured approximately 40 mm in length
(Figure). No erythema or drainage was noted. On palpation,
the lesion was firm and nonmobile, and sebum could not be
expressed. No punctum was identified. No other similar lesions
were found on the remainder of the skin exam, only benign nevi
and seborrheic keratoses.
The lesion was determined to be a single sebaceous nevus
based on texture, anatomic distribution, physical characteristics,
and presence for more than 5 years. A biopsy was not performed
as the lesion was not felt to have any significant characteristics
Proc (Bayl Univ Med Cent) 2017;30(2):211–212
Figure. A 40-mm linear sebaceous nevus in the left posterior auricular region.
suggesting malignancy. The patient was counseled to observe the
lesion for continued growth, change in texture, ulceration, or
pruritus. The lesion was photographed to assist with monitoring
it over time, with plans for yearly follow-ups.
DISCUSSION
Sebaceous glands are sebum-secreting glands located in the
dermis of all hair-bearing skin and are associated with hair follicles
and apocrine ducts (1). Hormones strongly affect the activity level
of these glands; thus, at the time of puberty, sebum production
increases. However, as an individual ages further, sebaceous glands
become diffusely hyperplastic, sebum production decreases, and
cell turnover and migration slow (2).
From Texas A&M College of Medicine (Kerwin, Menter), Baylor University Medical
Center at Dallas (Menter), and Texas Dermatology Associates, P.A., Dallas, Texas
(Menter).
Corresponding author: Blaire Kerwin, 4117 Wycliff Avenue, Dallas, TX 75219
(e-mail: [email protected]).
211
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