Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Epidermal Nevus Syndrome Medication

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: William D James, MD  more...
 
Updated: Jun 06, 2016
 

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.

Next

Vitamin D analogues

Class Summary

Vitamin D analogues may work by inhibiting epidermal proliferation, promoting keratinocyte differentiation, and/or exerting immunosuppressive effects on lymphoid cells.

Calcipotriol (Dovonex)

 

Calcipotriol is a synthetic vitamin D-3 analog that regulates skin cell production and development. It inhibits epidermal proliferation, promotes keratinocyte differentiation, and has immunosuppressive effects on lymphoid cells. Use 0.005% topical ointment.

Previous
 
Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Sergiusz Jozwiak, MD, PhD Professor and Head of Pediatric Neurology, Warsaw Medical University, Poland

Sergiusz Jozwiak, MD, PhD is a member of the following medical societies: Sigma Xi

Disclosure: Received honoraria from Novartis for speaking and teaching.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Van Perry, MD Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas School of Medicine at San Antonio

Van Perry, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Acknowledgements

Mark A Crowe, MD Assistant Clinical Instructor, Department of Medicine, Division of Dermatology, University of Washington School of Medicine

Mark A Crowe, MD is a member of the following medical societies: American Academy of Dermatology and North American Clinical Dermatologic Society

Disclosure: Nothing to disclose.

References
  1. Happle R. Gustav Schimmelpenning and the syndrome bearing his name. Dermatology. 2004. 209(2):84-7. [Medline].

  2. Mall V, Heinen F, Uhl M, Wellens E, Korinthenberg R. CNS lipoma in patients with epidermal nevus syndrome. Neuropediatrics. 2000 Aug. 31(4):175-9. [Medline].

  3. Altman J, Mehregan AH. Inflammatory linear verrucose epidermal nevus. Arch Dermatol. 1971 Oct. 104(4):385-9. [Medline].

  4. 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. 2004 Jul-Aug. 21(4):432-9. [Medline].

  5. Chantorn R, Shwayder T. Phacomatosis pigmentokeratotica: a further case without extracutaneous anomalies and review of the condition. Pediatr Dermatol. 2011 Nov-Dec. 28(6):715-9. [Medline].

  6. Groesser L, Herschberger E, Sagrera A, Shwayder T, Flux K, Ehmann L, et al. Phacomatosis Pigmentokeratotica Is Caused by a Postzygotic HRAS Mutation in a Multipotent Progenitor Cell. J Invest Dermatol. 2013 Jan 21. [Medline].

  7. Happle R. Linear Cowden nevus: a new distinct epidermal nevus. Eur J Dermatol. 2007 Mar-Apr. 17(2):133-6. [Medline].

  8. Bakhach M, Abbas O, Kibbi AG, Kurban M, Jundi MA. Nevoid hypertrichosis, diffuse lipoatrophy and epidermal nevus: a new syndrome?. Int J Dermatol. 2013 Mar 3. [Medline].

  9. Igawa S, Honma M, Minami-Hori M, Tsuchida E, Iizuka H, Ishida-Yamamoto A. Novel postzygotic KRAS mutation in a Japanese case of epidermal nevus syndrome presenting with two distinct clinical features, keratinocytic epidermal nevi and sebaceous nevi. J Dermatol. 2016 Jan. 43 (1):103-4. [Medline].

  10. 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. 2005 Dec 1. 139A(2):67-77. [Medline].

  11. 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. 2008 Sep 1. 146A(17):2275-9. [Medline].

  12. Ousager LB, Bygum A, Hafner C. Identification of a novel S249C FGFR3 mutation in a keratinocytic epidermal nevus syndrome. Br J Dermatol. 2012 Jan 9. [Medline].

  13. Farschtschi S, Mautner VF, Hollants S, Hagel C, Spaepen M, Schulte C, et al. Keratinocytic epidermal nevus syndrome with Schwann cell proliferation, lipomatous tumour and mosaic KRAS mutation. BMC Med Genet. 2015 Feb 10. 16(1):6. [Medline]. [Full Text].

  14. 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. 2010 Nov. 152A(11):2816-9. [Medline].

  15. Rogers M, McCrossin I, Commens C. Epidermal nevi and the epidermal nevus syndrome. A review of 131 cases. J Am Acad Dermatol. 1989 Mar. 20(3):476-88. [Medline].

  16. 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. 2005 May. 30(3):259-60. [Medline].

  17. Wu ZW, Shi WM, Sun Y, Li XJ, Song J. Cutaneous spindle cell squamous cell carcinoma in nevus sebaceous. Int J Dermatol. 2010 Dec. 49(12):1429-31. [Medline].

  18. Liu Y, Valdebran M, Chen J, Elbendary A, Wu F, Xu M. Nevus Sebaceous of Jadassohn With Eight Secondary Tumors of Follicular, Sebaceous, and Sweat Gland Differentiation. Am J Dermatopathol. 2016 Apr 19. [Medline].

  19. Miyagawa Y, Nakazawa M, Kudoh T. Epidermal nevus syndrome associated with anterior scleral staphyloma and ectopic bone and cartilaginous intraocular tissue. Jpn J Ophthalmol. 2010 Jan. 54(1):15-8. [Medline].

  20. Menni S, Boccardi D, Gualandri L. Keratinocytic epidermal nevus with oral involvement and cleft palate. G Ital Dermatol Venereol. 2008 Oct. 143(5):347-9. [Medline].

  21. 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. 2010 Jan. 152A(1):25-31. [Medline].

  22. Happle R. Epidermal nevus syndromes. Semin Dermatol. 1995 Jun. 14(2):111-21. [Medline].

  23. Mahto M, Ashworth J, Vickers DM. A case of linear epidermal naevus presenting as genital warts--a cautionary tale. Int J STD AIDS. 2005 Mar. 16(3):267-9. [Medline].

  24. 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. 2009 Feb 2. [Medline].

  25. Idriss MH, Elston DM. Secondary neoplasms associated with nevus sebaceus of Jadassohn: a study of 707 cases. J Am Acad Dermatol. 2014 Feb. 70(2):332-7. [Medline].

  26. Pernet C, Munoz J, Bessis D. [PENS (papular epidermal nevus with "skyline" basal cell layer)]. Ann Dermatol Venereol. 2015 Jan. 142(1):41-5. [Medline].

  27. 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. 2000 Apr. 22(2):108-18. [Medline].

  28. Micali G, Nasca MR, Musumeci ML. Effect of topical calcipotriol on inflammatory linear verrucous epidermal nevus. Pediatr Dermatol. 1995 Dec. 12(4):386-7. [Medline].

  29. Zvulunov A, Grunwald MH, Halvy S. Topical calcipotriol for treatment of inflammatory linear verrucous epidermal nevus. Arch Dermatol. 1997 May. 133(5):567-8. [Medline].

  30. Davison SP, Khachemoune A, Yu D, Kauffman LC. Nevus sebaceus of Jadassohn revisited with reconstruction options. Int J Dermatol. 2005 Feb. 44(2):145-50. [Medline].

  31. 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].

  32. Kiedrowicz M, Kacalak-Rzepka A, Królicki A, Maleszka R, Bielecka-Grzela S. Therapeutic effects of CO2 laser therapy of linear nevus sebaceous in the course of the Schimmelpenning-Feuerstein-Mims syndrome. Postepy Dermatol Alergol. 2013 Oct. 30(5):320-3. [Medline]. [Full Text].

  33. 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].

  34. Hato N, Tsujimura M, Takagi T, Okada M, Gyo K, Tohyama M, et al. Infantile inflammatory pseudotumor of the facial nerve as a complication of epidermal nevus syndrome with cholesteatoma. Auris Nasus Larynx. 2013 Feb 19. [Medline].

  35. Lefkowitz A, Schwartz RA, Lambert WC. Nevus comedonicus. Dermatology. 1999. 199(3):204-7. [Medline].

  36. Miteva LG, Dourmishev AL, Schwartz RA. Inflammatory linear verrucous epidermal nevus. Cutis. 2001 Nov. 68(5):327-30. [Medline].

Previous
Next
 
Characteristic epidermal nevus in the axillary fossa of a child with Jadassohn nevus phakomatosis.
An extensive plaque is observed over most of the left scapula, neck area, and lumbosacral location.
Plaque is evident in the region of the left groin, and it has a unilateral distribution.
Epidermal nevus syndrome, demonstrating extension of a plaque distally.
Extensive unilateral linear epidermal nevi in a 14-year-old girl with Jadassohn nevus phakomatosis. The plaques are elevated; some have verrucous characteristics.
An 8-year-old girl with Jadassohn nevus syndrome. Note typical plaques in the midline and on the arm and the neck. The plaques are darker and more verrucous on the arm and the neck than on the midline.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.