Generic selectors
Exact matches only
Search in title
Search in content
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Studies
Study Letter
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Reviews and Meta-analysis
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF

Translate this page into:

Letter To Editor
2008:74:5;513-515
doi: 10.4103/0378-6323.44326
PMID: 19052427

Iso-Kikuchi syndrome with absence of ring fingers and metacarpal bone abnormality

L Padmavathy1 , Lakshmana Rao2 , N Ethirajan3 , R Kanthimathi4 , M Adaikappan5
1 Department of Dermatology, Urban Health Center, Division of Community Medicine, Rajah Muthiah Medical College, Annamalai University, Chidambaram, Tamil Nadu, India
2 Department of Patholgy, Rajah Muthiah Medical College, Annamalai University, Chidambaram, Tamil Nadu, India
3 Department of Community Medicine, Rajah Muthiah Medical College, Annamalai University, Chidambaram, Tamil Nadu, India
4 Department of Orthopedics, Rajah Muthiah Medical College, Annamalai University, Chidambaram, Tamil Nadu, India
5 Department of Radiodiagnosis, Rajah Muthiah Medical College, Annamalai University, Chidambaram, Tamil Nadu, India

Correspondence Address:
L Padmavathy
B3, RSA Complex, Annamalai University, Annamalai Nagar - 608 002,Tamil Nadu
India
How to cite this article:
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;74:513-515
Copyright: (C)2008 Indian Journal of Dermatology, Venereology, and Leprology

Sir,

Iso-Kikuchi syndrome (I-K S), congenital onychodysplasia of the index fingers (COIF), is a rare condition characterized by various forms of nail dysplasia commonly involving the index fingers. Not infrequently, the neighboring fingers such as the middle fingers and thumbs are also affected. [1] A case of COIF in a 34-year-old female patient is reported here for its rarity.

A 34-year-old female patient, born of a consanguineous marriage, presented with generalized bodyache. On examination she was found to have 4 digits bilaterally and absent nail on the right index finger, while the left index finger showed a rudimentary nail. There was micronychia on the middle fingers bilaterally, being more marked on the right side [Figure - 1],[Figure - 2]. Lunula was absent in all nails. Palmar creases were normal. She could not flex her right index finger. All toe nails were normal. Her hair and mucous membranes were normal. She had normal dentition. Systemic examination did not reveal any abnormality. An X-ray of both the hands showed normal carpal bones and five metacarpal bones. The phalanges of the ring fingers were absent. The third and fourth metacarpal bones were found articulating with a single large proximal phalanx of the middle finger. Except for an elder brother, who had syndactyly bilaterally, her 3 other siblings were normal. Our patient had 2 children, who were unaffected. The patient gave a history that her mother had consumed an abortifacient while she was in utero.

Ichiro Kikuchi et al., [2] in 1974, coined the term ′congenital onychodysplasia of the index fingers′ (COIF) and identified a clinical syndrome consisting of nail dysplasias of the index fingers associated with underlying bone abnormalities. The first case report of this condition was by Kamei. Later, Iso collected a series of patients and defined the clinical syndrome. The term Iso-Kikuchi syndrome was introduced in 1980 by Robert Baran, a French dermatologist in Cannes. [1]

Iso-Kikuchi syndrome is rarely reported outside Japan, with an international incidence of 4.2 cases per 100,000 live births. [2]

The nails of COIF include the full spectrum of nail dysplasia, from irregular lunula, malalignment, micronychia (hypoplastic and rudimental), polyonychia (split rudimental), to anonychia, specifically affecting the index fingers. In our patient, there was micronychia of the middle fingers, while micronychia and malalignment of the left second toenail has been described by other authors. [3]

The five criteria characterizing COIF include the following: (i) congenital occurrence; (ii) unilateral or bilateral index finger involvement; (iii) variability in nail appearance; (iv) possible familial involvement; and (v) frequently associated bone abnormalities. [3] Our patient satisfied all the five of the above-mentioned criteria. The configuration of the lunula is supposed to play an important role in shaping the free edge of the nail plate. [4] In our patient, lunula was absent in all nails. Hemi-onychogryphosis of both index fingers and bifurcation of the distal phalanges are the other reported anomalies. [5] Our patient had two metacarpal bones articulating with the proximal phalanx of the middle finger, in addition to the absence of ring finger on both sides. The proximal phalanx of the middle finger was large and could be suggestive of syndactyly. The absence of alopecia and palmoplantar keratoderma and normal dentition ruled out ectodermal dysplasia.

In utero, ischemia of the palmar digital artery and a dysplastic change in the crescent-shaped cap of the distal phalanx are the two main candidate pathogenetic mechanisms that have been proposed. [6] Exposure to teratogens, especially antiepileptic drugs in utero, in mothers with epoxide hydrolase deficiency is another supposed cause of COIF. This probably could explain our patient′s predicament, as her mother had consumed an abortifacient when our patient was in utero. Due to patient′s noncompliance and lack of facilities, arteriographic studies could not be undertaken.

Iso-Kikuchi syndrome has also been associated with discoid lupus erythematous. As there were filiform arteries of the fingers and slow blood circulation on angiographic studies, the authors considered vascular pathogenic mechanisms to be responsible for this syndrome. [7] However, there was no clinical or laboratory evidence of any connective tissue disease in our patient.

Transmission of COIF can be either hereditary as autosomal dominant or sporadic. In our patient, a positive family history involving her brother could be suggestive of some hereditary involvement. Due to unavailability, chromosomal studies could not be undertaken.

Since COIF is only of cosmetic significance and has not interfered with her day-to-day activities, our patient was reassured and advised physiotherapy for the limitation of movements of the fingers.

The present case is being reported for its rarity and the hitherto unreported abnormality of the metacarpal bones, in addition to the absence of one digit (ring finger) on both sides.

References
1.
Baran R. Iso Kikuchi syndrome (C.O.I.F. syndrome): A report on 2 cases and a review of 44 cases in the literature. Ann Dermatol Venereol 1980;107:431-5.
[Google Scholar]
2.
Erickson K, Raugi GJ. Congenital onychodystrophy of the index fingers. e-Medicine 2007. p. 1-8.
[Google Scholar]
3.
Youn SH, Kwon OS, Park KC, Youn JI, Chung JH. Congenital onychodysplasia of the index fingers--Iso-Kikuchi syndrome; A case involving the second toenail. Clin Exp Dermatol 1996;21:457-8.
[Google Scholar]
4.
Baran R, Stroud JD. Congenital onychodysplasia of the index fingers: Iso and Kikuchi syndrome. Arch Dermatol 1984;120:243-4.
[Google Scholar]
5.
Morelli R, Tosti A, Patrizi A, Bardazzi F, Albisinni U. Iso and Kikuchi syndrome: Description of a case. G Ital Dermatol Venereol 1990;125:195-6.
[Google Scholar]
6.
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;98:330-5.
[Google Scholar]
7.
Bittar EQ, Parra CA, Ledesma de Prieto G, Briggs E, Ortiz Baeza O. Congenital ischemic onychodystrophy (Iso-Kikuchi syndrome) and chronic lupus erythematosus. Hautarzt 1988;39:750-2.
[Google Scholar]

Fulltext Views
164

PDF downloads
69
Show Sections