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Nevus sebaceous and nevus sebaceous syndrome

Teresa S Wright, MD, FAAD, FAAP
Section Editor
Moise L Levy, MD
Deputy Editor
Rosamaria Corona, MD, DSc


Nevus sebaceous, also called nevus sebaceous of Jadassohn or organoid nevus, is a benign hamartoma of the skin, characterized by hyperplasia of the epidermis, immature hair follicles, and sebaceous and apocrine glands. Lesions are usually present at birth and appear as waxy, yellow-orange or tan, hairless plaques (picture 2C). They have a tendency to thicken and become more verrucous over time, especially around the time of puberty [1].

Nevus sebaceous and the nevus sebaceous syndrome, also called Schimmelpenning syndrome, will be reviewed in this topic. The linear epidermal nevus and epidermal nevus syndrome are discussed separately. (See "Epidermal nevus and epidermal nevus syndrome".)


Nevus sebaceous occurs in approximately 0.3 percent of newborns, without sex predilection. It is usually sporadic, but familial cases have been reported [2,3].


Nevus sebaceous and nevus sebaceous syndrome (Schimmelpenning syndrome) are thought to be caused by postzygotic mosaic mutations in the HRAS or KRAS genes. RAS promotes cell growth through activation of multiple pathways, including the mitogen-activated protein kinase (MAPK) signal-transduction pathway. Activating mutations in this gene family are involved in the pathogenesis of several inherited malformation syndromes (eg, Costello syndrome, Noonan syndrome, neurofibromatosis 1), some of which are associated with an increased risk of cancer. (See "Causes of short stature", section on 'Noonan syndrome' and "Neurofibromatosis type 1 (NF1): Pathogenesis, clinical features, and diagnosis".)

An analysis of tissue samples from 65 individuals with nevus sebaceous and two with Schimmelpenning syndrome, found a HRAS c.37G>C mutation in 59 (91 percent) sebaceous nevi, but not in the adjacent normal epidermis [4]. A HRAS c.37G>C mutation and a KRAS c.35G>A mutation were found in lesional tissue samples from the two patients with nevus sebaceous syndrome. In another study, a HRAS c.37G>C mutation was found in a patient with Schimmelpenning syndrome and in 24 of 31 isolated sebaceous nevi but in none of six patient-matched normal skin samples [5]. In a whole-exome sequencing study of paired DNA from blood and nevus sebaceous tissue from five individuals with nevus sebaceous, recurrent somatic mutations in HRAS (p.Gly13Arg) and KRAS (p.Gly12Asp and p.Gly12Val) were identified in the epidermis and sebaceous lobules but not in the dermis or blood [6].


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Literature review current through: Jun 2017. | This topic last updated: Dec 07, 2015.
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