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Congenital Onychodystrophy of the Index Fingers Clinical Presentation

  • Author: Gregory J Raugi, MD, PhD; Chief Editor: William D James, MD  more...
 
Updated: Aug 11, 2014
 

History

Congenital onychodystrophy of the index fingers is congenital.

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Physical

Various nail abnormalities are observed, including (1) anonychia (lack of a fingernail), (2) micronychia (a small fingernail deviated to 1 side of the nail bed), (3) polyonychia (multiple, small, individual nails on one nail bed), (4) hemionychogryphosis (partial thickening and curvature of the fingernail), and (5) irregular lunula and nail malalignment. Appearance is displayed in the image below.

Micronychia of the left index finger and malalignmMicronychia of the left index finger and malalignment of the nail of the middle finger. Courtesy of Veterans Administration Puget Sound Health Care System.

Micronychia is the most common clinical manifestation of congenital onychodystrophy of the index finger. This differs from the usual form of micronychia, which is located on the central part of the finger. In the micronychia of congenital onychodystrophy of the index finger, the onychodysplasia is deviated to the radial aspect of the involved digits. Additionally, most commonly, the index finger is involved, either unilaterally or bilaterally. Involvement of other fingers and toes is also reported. A 2008 case report describes a patient with congenital onychodystrophy of the index finger who had metacarpal bone abnormalities and no ring finger.[11]

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Causes

The true cause of congenital onychodystrophy of the index finger remains obscure. However, some evidence supports hereditary transmission. Also possible is an unidentified in utero exposure to teratogens in genetically predisposed individuals, allowing expression of congenital onychodystrophy of the index finger. See Differentials below for similar conditions and distinguishing features.

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Contributor Information and Disclosures
Author

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.

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.

Jeffrey Meffert, MD Associate Clinical Professor of Dermatology, University of Texas School of Medicine at San Antonio

Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, 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: Nothing to disclose.

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.

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.

References
  1. Kikuchi I, Horikawa S, Amano F. Congenital onychodysplasia of the index fingers. Arch Dermatol. 1974 Nov. 110(5):743-6. [Medline].

  2. Kamei Y. A case of polynychia congenita sine polydactylia. Rinsho Dermatol. 1966. 8:956-8.

  3. Iso R. [Congenital nail defects of the index finger and reconstructive surgery]. Seikei Geka. 1969 Nov. 20(14):1383-4. [Medline].

  4. Millman AJ, Strier RP. Congenital onychodysplasia of the index fingers. Report of a family. J Am Acad Dermatol. 1982 Jul. 7(1):57-65. [Medline].

  5. 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. [Medline]. [Full Text].

  6. 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. [Medline].

  7. Kitayama Y, Tsukada S. Congenital onychodysplasia. Report of 11 cases. Arch Dermatol. 1983 Jan. 119(1):8-12. [Medline].

  8. 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. [Medline].

  9. Hussein TP, Brandt HR, Gabbi TV, Nico MM. Malformations of the index nails. Clin Exp Dermatol. 2008 Dec 15. [Medline].

  10. 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. [Medline].

  11. 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. [Medline].

  12. 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. [Medline].

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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.
 
 
 
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