Epidermal Nevus Syndrome Medication
- Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD more...
Medication Summary
No ideal medical therapy for the cutaneous lesions of epidermal nevus syndrome exists. The vitamin D analogue calcipotriol may be tried in each form of this disease, particularly inflammatory linear verrucous epidermal nevus.
Vitamin D analogues
Class Summary
May work by inhibiting epidermal proliferation, promoting keratinocyte differentiation, and/or exerting immunosuppressive effects on lymphoid cells.
Calcipotriol (Dovonex)
Synthetic vitamin D-3 analog that regulates skin cell production and development. Inhibits epidermal proliferation, promotes keratinocyte differentiation, and has immunosuppressive effects on lymphoid cells. Use 0.005% topical ointment.
Happle R. Gustav Schimmelpenning and the syndrome bearing his name. Dermatology. 2004;209(2):84-7. [Medline].
Mall V, Heinen F, Uhl M, Wellens E, Korinthenberg R. CNS lipoma in patients with epidermal nevus syndrome. Neuropediatrics. Aug 2000;31(4):175-9. [Medline].
Altman J, Mehregan AH. Inflammatory linear verrucose epidermal nevus. Arch Dermatol. Oct 1971;104(4):385-9. [Medline].
Vidaurri-de la Cruz H, Tamayo-Sanchez L, Duran-McKinster C, de la Luz Orozco-Covarrubias M, Ruiz-Maldonado R. Epidermal nevus syndromes: clinical findings in 35 patients. Pediatr Dermatol. Jul-Aug 2004;21(4):432-9. [Medline].
Chantorn R, Shwayder T. Phacomatosis pigmentokeratotica: a further case without extracutaneous anomalies and review of the condition. Pediatr Dermatol. Nov-Dec 2011;28(6):715-9. [Medline].
Happle R. Linear Cowden nevus: a new distinct epidermal nevus. Eur J Dermatol. Mar-Apr 2007;17(2):133-6. [Medline].
Heike CL, Cunningham ML, Steiner RD, et al. Skeletal changes in epidermal nevus syndrome: does focal bone disease harbor clues concerning pathogenesis?. Am J Med Genet A. Dec 1 2005;139A(2):67-77. [Medline].
Garcia-Vargas A, Hafner C, Perez-Rodriguez AG, et al. An epidermal nevus syndrome with cerebral involvement caused by a mosaic FGFR3 mutation. Am J Med Genet A. Sep 1 2008;146A(17):2275-9. [Medline].
Ousager LB, Bygum A, Hafner C. Identification of a novel S249C FGFR3 mutation in a keratinocytic epidermal nevus syndrome. Br J Dermatol. Jan 9 2012;[Medline].
Rijntjes-Jacobs EG, Lopriore E, Steggerda SJ, Kant SG, Walther FJ. Discordance for Schimmelpenning-Feuerstein-Mims syndrome in monochorionic twins supports the concept of a postzygotic mutation. Am J Med Genet A. Nov 2010;152A(11):2816-9. [Medline].
Rogers M, McCrossin I, Commens C. Epidermal nevi and the epidermal nevus syndrome. A review of 131 cases. J Am Acad Dermatol. Mar 1989;20(3):476-88. [Medline].
Ball EA, Hussain M, Moss AL. Squamous cell carcinoma and basal cell carcinoma arising in a naevus sebaceous of Jadassohn: case report and literature review. Clin Exp Dermatol. May 2005;30(3):259-60. [Medline].
Wu ZW, Shi WM, Sun Y, Li XJ, Song J. Cutaneous spindle cell squamous cell carcinoma in nevus sebaceous. Int J Dermatol. Dec 2010;49(12):1429-31. [Medline].
Miyagawa Y, Nakazawa M, Kudoh T. Epidermal nevus syndrome associated with anterior scleral staphyloma and ectopic bone and cartilaginous intraocular tissue. Jpn J Ophthalmol. Jan 2010;54(1):15-8. [Medline].
Menni S, Boccardi D, Gualandri L. Keratinocytic epidermal nevus with oral involvement and cleft palate. G Ital Dermatol Venereol. Oct 2008;143(5):347-9. [Medline].
Castori M, Annessi G, Castiglia D, et al. Systematized organoid epidermal nevus with eccrine differentiation, multiple facial and oral congenital scars, gingival synechiae, and blepharophimosis: a novel epidermal nevus syndrome. Am J Med Genet A. Jan 2010;152A(1):25-31. [Medline].
Happle R. Epidermal nevus syndromes. Semin Dermatol. Jun 1995;14(2):111-21. [Medline].
Mahto M, Ashworth J, Vickers DM. A case of linear epidermal naevus presenting as genital warts--a cautionary tale. Int J STD AIDS. Mar 2005;16(3):267-9. [Medline].
Wiedemeyer K, Hartschuh W. Trichoblastomas with Merkel cell proliferation in nevi sebacei in Schimmelpenning-Feuerstein-Mims syndrome - Histological differentiation between trichoblastomas and basal cell carcinomas. J Dtsch Dermatol Ges. Feb 2 2009;[Medline].
Jaqueti G, Requena L, Sanchez 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. Apr 2000;22(2):108-18. [Medline].
Micali G, Nasca MR, Musumeci ML. Effect of topical calcipotriol on inflammatory linear verrucous epidermal nevus. Pediatr Dermatol. Dec 1995;12(4):386-7. [Medline].
Zvulunov A, Grunwald MH, Halvy S. Topical calcipotriol for treatment of inflammatory linear verrucous epidermal nevus. Arch Dermatol. May 1997;133(5):567-8. [Medline].
Davison SP, Khachemoune A, Yu D, Kauffman LC. Nevus sebaceus of Jadassohn revisited with reconstruction options. Int J Dermatol. Feb 2005;44(2):145-50. [Medline].
Winston KR, Kang J, Laoprasert P, Kleinschmidt-DeMasters BK. Hemispherectomy in a premature neonate with linear sebaceous nevus syndrome. Pediatr Neurosurg. 2008;44(2):159-64. [Medline].
Shahgholi E, Mollaian M, Haghshenas Z, Honarmand M. Congenital rhabdomyosarcoma, central precocious puberty, hemihypertrophy and hypophosphatemic rickets associated with epidermal nevus syndrome. J Pediatr Endocrinol Metab. 2011;24(11-12):1063-6. [Medline].
Adrian RM, Baden HP. Analysis of epidermal fibrous proteins in inflammatory linear verrucous epidermal nevus. Arch Dermatol. Oct 1980;116(10):1179-80. [Medline].
al-Fouzan AS, Hassab-el-Naby HM, Nanda A. Congenital linear psoriasis: a case report. Pediatr Dermatol. Dec 1990;7(4):303-6. [Medline].
Allison MA, Dunn CL, Pedersen RC. What syndrome is this? Epidermal nevus syndrome: a neurologic variant with hemimegalencephaly, facial hemihypertrophy and gyral malformation. Pediatr Dermatol. Jan-Feb 1998;15(1):59-61. [Medline].
Bazopoulou-Kyrkanidou E, Alexandridis C, Tosios KI, Sotiriadou S, Angelopoulos AP. Epidermal nevus syndrome with development of a mandibular ameloblastoma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. Jul 2000;90(1):64-70. [Medline].
Bernhard JD, Owen WR, Steinman HK, Kaplan LA, Menkes AB, Baden HP. Inflammatory linear verrucous epidermal nevus. Epidermal protein analysis in four patients. Arch Dermatol. Feb 1984;120(2):214-5. [Medline].
Bittar M, Happle R. CHILD syndrome avant la lettre. J Am Acad Dermatol. Feb 2004;50(2 Suppl):S34-7. [Medline].
Canyigit M, Oguz KK. Epidermal nevus syndrome with internal carotid artery occlusion and intracranial and orbital lipomas. AJNR Am J Neuroradiol. Aug 2006;27(7):1559-61. [Medline].
Caux F, Plauchu H, Chibon F, et al. Segmental overgrowth, lipomatosis, arteriovenous malformation and epidermal nevus (SOLAMEN) syndrome is related to mosaic PTEN nullizygosity. Eur J Hum Genet. Jul 2007;15(7):767-73. [Medline].
Crovato F, De Marchi R, Nazzari G, Desirello G, Giunchedi M. [Schimmelpenning-Feuerstein-Mims syndrome, a variant of the epidermal nevus syndrome]. G Ital Dermatol Venereol. Jul-Aug 1983;118(4):211-4. [Medline].
Domanska-Pakiela D, Jozwiak S. Modern opinions on etiopathogenesis, diagnosis and treatment of Jadassohn nevus syndrome. Ped Polska. 1998;73:1297-1301.
Farajzadeh S, Zahedi MJ, Moghaddam SD. A new gastrointestinal finding in Proteus syndrome: report of a case of multiple colonic hemangiomas. Int J Dermatol. Feb 2006;45(2):135-8. [Medline].
Fink-Puches R, Soyer HP, Pierer G, Kerl H, Happle R. Systematized inflammatory epidermal nevus with symmetrical involvement: an unusual case of CHILD syndrome?. J Am Acad Dermatol. May 1997;36(5 Pt 2):823-6. [Medline].
Fitzpatrick TB, Mihm MC. Multiple melanocytoma. Arch Dermatol. Jul 1970;102(1):126. [Medline].
Gobello T, Mazzanti C, Zambruno G, Chinni LM, Happle R. New type of epidermal nevus syndrome. Dermatology. 2000;201(1):51-3. [Medline].
Golitz LE, Weston WL. Inflammatory linear verrucous epidermal nevus. Association with epidermal nevus syndrome. Arch Dermatol. Oct 1979;115(10):1208-9. [Medline].
Happle R. How many epidermal nevus syndromes exist? A clinicogenetic classification. J Am Acad Dermatol. Sep 1991;25(3):550-6. [Medline].
Hodge JA, Ray MC, Flynn KJ. The epidermal nevus syndrome. Int J Dermatol. Feb 1991;30(2):91-8. [Medline].
Hsu S, Nikko A. Unilateral atrophic skin lesion with features of atrophoderma vermiculatum: a variant of the epidermal nevus syndrome?. J Am Acad Dermatol. Aug 2000;43(2 Pt 1):310-2. [Medline].
Ivker R, Resnick SD, Skidmore RA. Hypophosphatemic vitamin D-resistant rickets, precocious puberty, and the epidermal nevus syndrome. Arch Dermatol. Dec 1997;133(12):1557-61. [Medline].
Jozwiak S, Domanska-Pakiela D, Potakiewicz W. Clinical observations of Jadassohn nevus syndrome in 7 children. Ped Polska. 1998;73:1273-1278.
Jurecka W, Neumann RA, Knobler RM. Porokeratoses: immunohistochemical, light and electron microscopic evaluation. J Am Acad Dermatol. Jan 1991;24(1):96-101. [Medline].
Kawaguchi H, Takeuchi M, Ono H, Nakajima H. Adult onset of inflammatory linear verrucous epidermal nevus. J Dermatol. Sep 1999;26(9):599-602. [Medline].
Kishida ES, Muniz Silva MA, da Costa Pereira F, Sanches JA Jr, Sotto MN. Epidermal nevus syndrome associated with adnexal tumors, spitz nevus, and hypophosphatemic vitamin D-resistant rickets. Pediatr Dermatol. Jan-Feb 2005;22(1):48-54. [Medline].
Lefkowitz A, Schwartz RA, Lambert WC. Nevus comedonicus. Dermatology. 1999;199(3):204-7. [Medline].
Menascu S, Donner EJ. Linear nevus sebaceous syndrome: case reports and review of the literature. Pediatr Neurol. Mar 2008;38(3):207-10. [Medline].
Micali G, Nasca MR, De Pasquale R. Linear acantholytic dyskeratotic epidermal nevus of the sole. Pediatr Dermatol. Mar-Apr 1999;16(2):166-8. [Medline].
Mikulska D, Nowak A. A case of Darier-like epidermal nevus. Postepy Dermatol (Poznan). 2000;17:347-350.
Miteva LG, Dourmishev AL, Schwartz RA. Inflammatory linear verrucous epidermal nevus. Cutis. Nov 2001;68(5):327-30. [Medline].
Ogunbiyi AO, Ogunbiyi JO. Nevus depigmentosus and inflammatory linear epidermal nevus--an unusual combination with a note on histology. Int J Dermatol. Aug 1998;37(8):600-2. [Medline].
Pandhi D, Reddy BS. A rare association of epidermal nevus syndrome and ainhum-like digital constrictions. Pediatr Dermatol. Jul-Aug 2002;19(4):349-52. [Medline].
Parano E, Rizzo R, Micali G, et al. Woolly hair nevus and linearepidermal nevus: a clinical association. Giornale Int Dermatol Pediatr. 1993;5:73-6.
Pavone L, Curatolo P, Rizzo R, et al. Epidermal nevus syndrome: a neurologic variant with hemimegalencephaly, gyral malformation, mental retardation, seizures, and facial hemihypertrophy. Neurology. Feb 1991;41(2 (Pt 1)):266-71. [Medline].
Perez Chavarria EL, Castellanos E, Linares L. Sindrome del nevo sebaceo. Med Cutanea. 1996;24:73-5.
Piper HF, Bastian GO. [Epidermal nevus syndrome (Schimmelpenning-Feuerstein-Mims syndrome) and early childhood cataract development]. Klin Monatsbl Augenheilkd. Apr 1983;182(4):318-21. [Medline].
Prayson RA, Kotagal P, Wyllie E, Bingaman W. Linear epidermal nevus and nevus sebaceus syndromes: a clinicopathologic study of 3 patients. Arch Pathol Lab Med. Apr 1999;123(4):301-5. [Medline].
Rahbari H, Cordero AA, Mehregan AH. Linear porokeratosis. A distinctive clinical variant of porokeratosis of Mibelli. Arch Dermatol. Apr 1974;109(4):526-8. [Medline].
Rogers M. Epidermal nevi and the epidermal nevus syndromes: a review of 233 cases. Pediatr Dermatol. Dec 1992;9(4):342-4. [Medline].
Saedi T, Cetas J, Chang R, Krol A, Selden NR. Newborn with sebaceous nevus of jadassohn presenting as exophytic scalp lesion. Pediatr Neurosurg. 2008;44(2):144-7. [Medline].
Sasaki M, Matsuda H, Arai Y, Hashimoto T. Startle-induced epilepsy in a patient with epidermal nevus syndrome. Pediatr Neurol. Apr 1998;18(4):346-9. [Medline].
Schepis C, Greco D, Siragusa M, Batolo D, Romano C. Rubinstein-Taybi syndrome with epidermal nevus: a case report. Pediatr Dermatol. Jan-Feb 2001;18(1):34-7. [Medline].
Solomon LM. Epidermal nevus syndrome. Mod Probl Paediatr. 1975;17:27-30. [Medline].
Stein KM, Shmunes E, Thew M. Neurofibromatosis presenting as the epidermal nevus syndrome. Arch Dermatol. Feb 1972;105(2):229-32. [Medline].
Surve TY, Muranjan MN, Deshmukh CT, Warke CS, Bharucha BA. Inflammatory linear verrucous epidermal nevus syndrome with bilateral vertebral artery occlusion. Indian Pediatr. Aug 1999;36(8):820-3. [Medline].
Thivolet J, Goujon C. Linear psoriasis and systematized epidermolytic nevus. Arch Dermatol. Apr 1982;118(4):285-6. [Medline].
Waltz KM, Helm KF, Billingsley EM. The spectrum of epidermal nevi: a case of verrucous epidermal nevus contiguous with nevus sebaceus. Pediatr Dermatol. May-Jun 1999;16(3):211-3. [Medline].
Welch ML, Smith KJ, Skelton HG, et al. Immunohistochemical features in inflammatory linear verrucous epidermal nevi suggest a distinctive pattern of clonal dysregulation of growth. Military Medical Consortium for the Advancement of Retroviral Research. J Am Acad Dermatol. Aug 1993;29(2 Pt 1):242-8. [Medline].
Yu TW, Tsau YK, Young C, Chiu HC, Shen YZ. Epidermal nevus syndrome with hypermelanosis and chronic hyponatremia. Pediatr Neurol. Feb 2000;22(2):151-4. [Medline].
Zaremba J. Jadassohn's naevus phakomatosis: 2. A study based on a review of thirty-seven cases. J Ment Defic Res. Jun 1978;22(2):103-23. [Medline].
Zgrzyblowski A, Ryc K, Paradecki W. [Inflammatory linear verrucous epidermal naevus (Ilven)]. Przegl Dermatol. Jan-Feb 1988;75(1):46-9. [Medline].

