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Nevus sebaceus - Hair and Scalp
See also in: Overview
Other Resources UpToDate PubMed

Nevus sebaceus - Hair and Scalp

See also in: Overview
Contributors: David O'Connell MD, Lauren Ko, Belinda Tan MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Nevus sebaceus (of Jadassohn) is a common congenital hamartoma that is usually found on the scalp. It is considered a subtype of organoid epidermal nevus. In adulthood, it usually appears as a solitary, hairless, well-demarcated, yellow-tan plaque with a velvety or verrucoid surface, ranging in size from a few millimeters to a few centimeters. Lesions have commonly been present since birth, undergo flattening during childhood, and become elevated again during puberty. Most lesions are sporadic, with rare familial forms reported. There is no ethnic or sex predilection.

Nevus sebaceus may be found on the face or neck, and less frequently, the torso or extremities may be affected. It may be multifocal following the lines of Blaschko. Extensive or multifocal lesions may be associated with the nevus sebaceus syndrome (also known as the sebaceous nevus syndrome; Schimmelpenning syndrome), where it is accompanied by neurological, ocular, cardiovascular, skeletal, and urologic abnormalities. Phakomatosis pigmentokeratotica is the presence of a speckled lentiginous nevus and a linear nevus sebaceus.

In late adolescence through adulthood, numerous secondary neoplasms may arise in a nevus sebaceus. This occurs in a small percentage of cases. They are mostly benign adnexal tumors such as trichoblastoma and syringocystadenoma papilliferum. Trichilemmomas, eccrine poromas, and sebaceomas have also developed. The most commonly encountered malignancy to arise within nevus sebaceus is basal cell carcinoma (BCC). The frequency of BCC in nevus sebaceus is thought to have been previously over-reported, with most BCC cases having been reinterpreted as trichoblastomas. Squamous cell carcinoma and sebaceous carcinoma develop very rarely, and a case each of microcystic adnexal carcinoma and melanoma has been reported.

Codes

ICD10CM:
D23.9 – Other benign neoplasm of skin, unspecified

SNOMEDCT:
707136009 – Nevus sebaceous

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

In children and infants, also consider:
  • Solitary mastocytoma – favors distal extremities, no alopecia and positive Darier sign
  • Juvenile xanthogranuloma – smooth, dome-shaped papule or nodule
  • Perinatal trauma to the scalp from forceps, scalp blood sampling, or monitor electrode placement may result in scarring with associated alopecia.
  • Aplasia cutis congenita presents at birth as a focal erosion or ulceration in the scalp. Lesions eventually heal with atrophy and scarring with associated alopecia. A ring of long, coarse, dark hair (hair collar sign) suggests an associated underlying neural tube closure defect.

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed:02/13/2020
Last Updated:10/11/2022
Copyright © 2022 VisualDx®. All rights reserved.
Nevus sebaceus - Hair and Scalp
See also in: Overview
A medical illustration showing key findings of Nevus sebaceus : Face, Neck, Present at birth, Scalp, Verrucous scaly plaque, Single skin lesion
Clinical image of Nevus sebaceus - imageId=79068. Click to open in gallery.  caption: 'A light brown and yellowish cobblestone plaque with a linear configuration on the lateral cheek.'
A light brown and yellowish cobblestone plaque with a linear configuration on the lateral cheek.
Copyright © 2022 VisualDx®. All rights reserved.