Epidermal nevi(EN)represent a heterogeneous group of mosaic skin lesions frequently following the lines of Blaschko. They are divided into organoid and nonorganoid types. Herein we report on a 35-year-old man with a rather unusual type of EN. A linear lesion involving the trunk and following Blaschko’s lines was conspicuously bordered by dark brown papules. The flat reddish central area histopathologically showed sebaceous hyperplasia consistent with nevus sebaceus, an organoid EN type, whereas the elevated margin showed features of a common nonorganoid keratinocytic EN.For this peculiar disorder, we propose the term ‘nevus marginatus’. So far it is not clear whether nevus marginatus represents a distinct entity or merely an unusual clinical variant of nevus sebaceus.

Keywords:
Congenital, Epidermal nevus, Sebaceous gland
1.
Happle R, Rogers M: Epidermal nevi. Adv Dermatol 2002;18:175–201.
2.
Happle R: Epidermal nevus syndromes. Semin Dermatol 1995;14:111–121.
3.
Waltz KM, Helm KF, Billingsley EM: The spectrum of epidermal nevi: a case of verrucous epidermal nevus contiguous with nevus sebaceus. Pediatr Dermatol 1999;16:211–213.
4.
Happle R: What is a nevus? A proposed definition of a common medical term. Dermatology 1995;191:1–5.
5.
Happle R: Mosaicism in human skin: understanding the patterns and mechanisms. Arch Dermatol 1993;129:1460–1470.
6.
van Oers JM, Lurkin I, van Exsel AJ, et al: A simple and fast method for the simultaneous detection of nine fibroblast growth factor receptor 3 mutations in bladder cancer and voided urine. Clin Cancer Res 2005;11:7743–7748.
7.
Hafner C, van Oers JM, Vogt T, et al: Mosaicism of activating FGFR3 mutations in human skin causes epidermal nevi. J Clin Invest 2006;116:2201–2207.
8.
Tevar E, Torrelo A, Contreras F, Colmenero I, Zambrano A: Multiple basal cell carcinomas on phacomatosis pigmentokeratotica. Actas Dermosifiliogr 2006;97:518–521.
9.
Solomon LM, Esterly NB: Epidermal and other congenital organoid nevi. Curr Probl Pediatr 1975;6:1–56.
10.
Solomon LM, Fretzin DF, Dewald RL: The epidermal nevus syndrome. Arch Dermatol 1968;97:273–285.
11.
Hodge JA, Ray MC, Flynn KJ: The epidermal nevus syndrome. Int J Dermatol 1991;30:91–98.
12.
Happle R: How many epidermal nevus syndromes exist? A clinicogenetic classification. J Am Acad Dermatol 1991;25:550–556.
13.
Rothnagel JA, Dominey AM, Dempsey LD, et al: Mutations in the rod domains of keratins 1 and 10 in epidermolytic hyperkeratosis. Science 1992;257:1128–1130.
14.
Paller AS, Syder AJ, Chan YM, et al: Genetic and clinical mosaicism in a type of epidermal nevus. N Engl J Med 1994;331:1408–1415.
15.
Konig A, Happle R, Bornholdt D, Engel H, Grzeschik KH: Mutations in the NSDHL gene, encoding a 3beta-hydroxysteroid dehydrogenase, cause CHILD syndrome. Am J Med Genet 2000;90:339–346.
16.
Xin H, Matt D, Qin JZ, Burg G, Boni R: The sebaceous nevus: a nevus with deletions of the PTCH gene. Cancer Res 1999;59:1834–1836.
17.
Takata M, Tojo M, Hatta N, Ohara K, Yamada M, Takehara K: No evidence of deregulated patched-hedgehog signaling pathway in trichoblastomas and other tumors arising within nevus sebaceus. J Invest Dermatol 2001;117:1666–1670.
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2008
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