Approach Considerations

Aggressive surgical treatment is generally not warranted. Reassurance and support are important to help affected individuals adjust to living with this disorder.

Medical Care

Congenital onychodystrophy of the index finger (COIF) is of only cosmetic significance. After diagnosis, reassuring the patient and family is all that is necessary. Keeping nails clipped short can be of value to ensure that nails do not catch on surfaces during activities of daily living. Artificial nails can be a useful camouflage in self-conscious individuals.^[18]

Surgical Care

Surgical correction may be elected in cases in which syndactyly is present. Pincer nail deformity can be successfully treated with a specialized nail device that helps address nail curvature.^[19]Use of a device composed of a shape-memory alloy can help change the nail shape and have a lasting benefit as a new nail grows in.

Consultations

No consultations are necessary in onychodystrophy of the index finger (COIF).

Diet

Congenital onychodystrophy of the index finger (COIF) is not related to or affected by diet.

Activity

The absence of an index fingernail rarely affects the patient's ability to perform functions. Often, the middle fingernail can be used with the thumb if a pincer grip is required. Occasionally, the index finger may be functionally impaired.

Medication

Kikuchi I, Horikawa S, Amano F. Congenital onychodysplasia of the index fingers. Arch Dermatol. 1974 Nov. 110(5):743-6. [QxMD MEDLINE Link].
Kamei Y. A case of polynychia congenita sine polydactylia. Rinsho Dermatol. 1966. 8:956-8.
Iso R. [Congenital nail defects of the index finger and reconstructive surgery]. Seikei Geka. 1969 Nov. 20(14):1383-4. [QxMD MEDLINE Link].
Millman AJ, Strier RP. Congenital onychodysplasia of the index fingers. Report of a family. J Am Acad Dermatol. 1982 Jul. 7(1):57-65. [QxMD MEDLINE Link].
Raykova D, Klar J, Azhar A, Khan TN, Malik NA, Iqbal M, et al. Autosomal recessive transmission of a rare KRT74 variant causes hair and nail ectodermal dysplasia: allelism with dominant woolly hair/hypotrichosis. PLoS One. 2014. 9(4):e93607. [QxMD MEDLINE Link]. [Full Text].
Kantaputra P, Kaewgahya M, Jotikasthira D, Kantaputra W. Tricho-odonto-onycho-dermal dysplasia and WNT10A mutations. Am J Med Genet A. 2014 Apr. 164A(4):1041-8. [QxMD MEDLINE Link].
Kitayama Y, Tsukada S. Congenital onychodysplasia. Report of 11 cases. Arch Dermatol. 1983 Jan. 119(1):8-12. [QxMD MEDLINE Link].
Franceschini P, Licata D, Guala A, Di Cara G, Franceschini D. Peculiar facial appearance and generalized brachydactyly in a patient with congenital onychodysplasia of the index fingers (Iso-Kikuchi syndrome). Am J Med Genet. 2001 Feb 1. 98(4):330-5. [QxMD MEDLINE Link].
Matsukura H, Oose Y, Yuki H. Neonate born to hepatitis B carrier mother presenting with congenital onychodysplasia of the index finger (Iso-Kikuchi syndrome). Turk J Pediatr. 2014 Jul-Aug. 56 (4):462-3. [QxMD MEDLINE Link].
L'Orphelin JM, Picard C, Andre J, Richert B, Dompmartin A. [Iso Kikuchi syndrome: Congenital onychodysplasia of the index fingers, or COIF syndrome]. Ann Dermatol Venereol. 2018 Apr. 145 (4):304-306. [QxMD MEDLINE Link].
Hussein TP, Brandt HR, Gabbi TV, Nico MM. Malformations of the index nails. Clin Exp Dermatol. 2008 Dec 15. [QxMD MEDLINE Link].
Prais D, Horev G, Merlob P. Prevalence and new phenotypic and radiologic findings in congenital onychodysplasia of the index finger. Pediatr Dermatol. 1999 May-Jun. 16(3):201-4. [QxMD MEDLINE Link].
Padmavathy L, Rao L, Ethirajan N, Kanthimathi R, Adaikappan M. Iso-Kikuchi syndrome with absence of ring fingers and metacarpal bone abnormality. Indian J Dermatol Venereol Leprol. 2008 Sep-Oct. 74(5):513-5. [QxMD MEDLINE Link].
Crowe D, DiSano K. Congenital onychodysplasia of the index finger presenting as a congenital bifid nail. Dermatol Online J. 2014 Nov 15. 20 (11):[QxMD MEDLINE Link].
Asai Y, Irvine AD. Ectodermal Dysplasias. In: Irvine AD, Hoeger PH, Yan AC, eds. Harper's Textbook of Pediatric Dermatology. 3rd edition. UK: Wiley-Blackwell; 2011. 127.88-127.95.
Szabo L, Kocsis K, Hollody K, Soroncz M, Erwa W, Andits M. [DOOR (deafness, onychodystrophy, osteodystrophy, mental retardation) syndrome]. Orv Hetil. 2004 May 30. 145(22):1183-7. [QxMD MEDLINE Link].
Choi JY, Kim SE, Lee SE, Kim SC. Olmsted Syndrome Caused by a Heterozygous p.Gly568Val Missense Mutation in TRPV3 Gene. Yonsei Medical Journal. 2018 Mar. 59:341-344. [QxMD MEDLINE Link].
Crowe D, DiSano K. Congenital onychodysplasia of the index finger presenting as a congenital bifid nail. Dermatol Online J. 2014 Nov 15. 20 (11):[QxMD MEDLINE Link].
Park SW, Lee DY. Treatment of congenital onychodysplasia of the index finger with specialized nail device. Clin Exp Dermatol. 2013 Oct. 38 (7):791-2. [QxMD MEDLINE Link].

Media Gallery

Micronychia of the left index finger and malalignment of the nail of the middle finger. Courtesy of Veterans Administration Puget Sound Health Care System.
Y-shaped bifurcation of the distal phalanx of the left index finger in the lateral view. Courtesy of Veterans Administration Puget Sound Health Care System.
Y-shaped bifurcation of the distal phalanx of the right index finger in the lateral view. Courtesy of Veterans Administration Puget Sound Health Care System.
This infant illustrates the typical presentation of congenital onychodysplasia of the index finger (COIF). Examination of the right index finger reveals micronychia. The changes of the left index finger approach anonychia except for the presence of a small amount of dystrophic nail. Courtesy of Dr. Ilene Rothman.

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Author

Stefanos F Haddad, MD Fellow in Hand and Upper Extremity Surgery, Rothman Institute

Stefanos F Haddad, MD is a member of the following medical societies: American Academy of Orthopaedic Surgeons, American Society for Surgery of the Hand, New York State Society of Orthopaedic Surgeons

Disclosure: Nothing to disclose.

Coauthor(s)

Thomas N Helm, MD Clinical Professor of Dermatology and Pathology, University of Buffalo, State University of New York School of Medicine and Biomedical Sciences; Director, Buffalo Medical Group Dermatopathology Laboratory

Thomas N Helm, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society of Dermatopathology

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Former 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: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

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: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

Additional Contributors

Richard K Scher, MD Adjunct Professor of Dermatology, University of North Carolina at Chapel Hill School of Medicine; Professor Emeritus of Dermatology, Columbia University College of Physicians and Surgeons

Richard K Scher, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American Medical Association, Association of Military Surgeons of the US, International Society for Dermatologic Surgery, Noah Worcester Dermatological Society, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Gregory J Raugi, MD, PhD Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle School of Medicine; Chief, Dermatology Section, Primary and Specialty Care Service, Veterans Administration Medical Center of Seattle

Gregory J Raugi, MD, PhD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Kristin Erickson, MD, to the development and writing of this article.