eMedicine Specialties > Dermatology > Pediatric Diseases

Piebaldism: Follow-up

Author: Michael D Fox, MD, Attending Physician, Department of Emergency Medicine, Marin General Hospital
Coauthor(s): Camila K Janniger, MD, Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, New Jersey Medical School
Contributor Information and Disclosures

Updated: Jan 30, 2009

Follow-up

Prognosis

  • Piebaldism is a benign disorder. However, patients are at risk for actinic complications related to absence of cutaneous melanocytes.

Patient Education

  • Educate patients about the genetic transmission of the disorder.
  • Educate patients about use of sunscreens, sunprotective measures (eg, wide-brimmed hat, long-sleeved shirts, long pants), sun avoidance during peak hours of ultraviolet exposure during the day, and self-examinations because recurrent sun damage may result in an increased risk of cutaneous malignancy.
  • Patients who are self-conscious about the appearance of their skin may benefit from use of a camouflage cover-up, such as Dermablend or other similar camouflage measures.
 


More on Piebaldism

Overview: Piebaldism
Differential Diagnoses & Workup: Piebaldism
Treatment & Medication: Piebaldism
Follow-up: Piebaldism
Multimedia: Piebaldism
References

References

  1. Ezoe K, Holmes SA, Ho L, et al. Novel mutations and deletions of the KIT (steel factor receptor) gene in human piebaldism. Am J Hum Genet. Jan 1995;56(1):58-66. [Medline].

  2. Richards KA, Fukai K, Oiso N, Paller AS. A novel KIT mutation results in piebaldism with progressive depigmentation. J Am Acad Dermatol. Feb 2001;44(2):288-92. [Medline].

  3. Tosaki H, Kunisada T, Motohashi T, Aoki H, Yoshida H, Kitajima Y. Mice transgenic for Kit(V620A): recapitulation of piebaldism but not progressive depigmentation seen in humans with this mutation. J Invest Dermatol. May 2006;126(5):1111-8. [Medline].

  4. Spritz RA. "Out, damned spot!". J Invest Dermatol. May 2006;126(5):949-51. [Medline].

  5. Jan IA, Stroedter L, Haq AU, Din ZU. Association of Shah-Waardenburgh syndrome: a review of 6 cases. J Pediatr Surg. Apr 2008;43(4):744-7. [Medline].

  6. Ruan HB, Zhang N, Gao X. Identification of a novel point mutation of mouse proto-oncogene c-kit through N-ethyl-N-nitrosourea mutagenesis. Genetics. Feb 2005;169(2):819-31. [Medline].

  7. Chow RK, Stewart WD, Ho VC. Graft-versus-host reaction affecting lesional skin but not normal skin in a patient with piebaldism. Br J Dermatol. Jan 1996;134(1):134-7. [Medline].

  8. Matsunaga H, Tanioka M, Utani A, Miyachi Y. Familial case of piebaldism with regression of white forelock. Clin Exp Dermatol. Jul 2008;33(4):511-2. [Medline].

  9. Tamayo ML, Gelvez N, Rodriguez M, Florez S, Varon C, Medina D, et al. Screening program for Waardenburg syndrome in Colombia: clinical definition and phenotypic variability. Am J Med Genet A. Apr 15 2008;146A(8):1026-31. [Medline].

  10. Njoo MD, Nieuweboer-Krobotova L, Westerhof W. Repigmentation of leucodermic defects in piebaldism by dermabrasion and thin split-thickness skin grafting in combination with minigrafting. Br J Dermatol. Nov 1998;139(5):829-33. [Medline].

  11. Garg T, Khaitan BK, Manchanda Y. Autologous punch grafting for repigmentation in piebaldism. J Dermatol. Nov 2003;30(11):849-50. [Medline].

  12. Desch LW. White forelock could be early sign of tuberous sclerosis. Arch Pediatr Adolesc Med. Jun 1996;150(6):651-2. [Medline].

  13. Falabella R, Barona M, Escobar C, Borrero I, Arrunategui A. Surgical combination therapy for vitiligo and piebaldism. Dermatol Surg. Oct 1995;21(10):852-7. [Medline].

  14. Farag TI, el-Ramly MA, Sakr MF. A Bedouin kindred with 19 piebalds in 5 generations. Clin Genet. Dec 1992;42(6):326-8. [Medline].

  15. Janjua SA, Khachemoune A, Guldbakke KK. Piebaldism: a case report and a concise review of the literature. Cutis. Nov 2007;80(5):411-4. [Medline].

  16. Murakami T, Fukai K, Oiso N. New KIT mutations in patients with piebaldism. J Dermatol Sci. Jun 2004;35(1):29-33. [Medline].

  17. Murakami T, Hosomi N, Oiso N, et al. Analysis of KIT, SCF, and initial screening of SLUG in patients with piebaldism. J Invest Dermatol. Mar 2005;124(3):670-2. [Medline].

  18. Nomura K, Hatayama I, Narita T, Kaneko T, Shiraishi M. A novel KIT gene missense mutation in a Japanese family with piebaldism. J Invest Dermatol. Aug 1998;111(2):337-8. [Medline].

  19. Spritz RA. Molecular basis of human piebaldism. J Invest Dermatol. Nov 1994;103(5 Suppl):137S-140S. [Medline].

  20. Spritz RA. Piebaldism, Waardenburg syndrome, and related disorders of melanocyte development. Semin Cutan Med Surg. Mar 1997;16(1):15-23. [Medline].

  21. Spritz RA, Beighton P. Piebaldism with deafness: molecular evidence for an expanded syndrome. Am J Med Genet. Jan 6 1998;75(1):101-3. [Medline].

  22. Syrris P, Malik NM, Murday VA, Patton MA, et al. Three novel mutations of the proto-oncogene KIT cause human piebaldism. Am J Med Genet. Nov 6 2000;95(1):79-81. [Medline].

  23. Thomas I, Kihiczak GG, Fox MD, Janniger CK, Schwartz RA. Piebaldism: an update. Int J Dermatol. Oct 2004;43(10):716-9. [Medline].

  24. Ward KA, Moss C, Sanders DS. Human piebaldism: relationship between phenotype and site of kit gene mutation. Br J Dermatol. Jun 1995;132(6):929-35. [Medline].

Further Reading

Keywords

piebaldism, partial albinism, familial white spotting, white forelock, poliosis circumscriptum, Waardenburg syndrome, KIT proto-oncogene

Contributor Information and Disclosures

Author

Michael D Fox, MD, Attending Physician, Department of Emergency Medicine, Marin General Hospital
Michael D Fox, MD is a member of the following medical societies: American Academy of Emergency Medicine, American Academy of Family Physicians, and California Medical Association
Disclosure: Nothing to disclose.

Coauthor(s)

Camila K Janniger, MD, Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, New Jersey Medical School
Camila K Janniger, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Medical Editor

Albert C Yan, MD, Section Chief, Associate Professor, Department of Pediatrics, Section of Dermatology, Children's Hospital of Philadelphia and University of Pennsylvania
Albert C Yan, MD is a member of the following medical societies: American Academy of Dermatology, American Academy of Pediatrics, Society for Investigative Dermatology, and Society for Pediatric Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

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