Onychodysplasia congenital of the index finger Q84.6

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 27.11.2022

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Synonym(s)

congenital ischemic onychodystrophy; congenital onychodysplasia; Congenital syndrome of the index finger; Iso-Kikuchi Syndrome; Onychodystrophy congenital ischemic

History
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Iso, 1969; Kikuchi, 1974

Definition
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Rare, congenital, very variable nail dysplasia, mainly of the index finger, often associated with bony changes of the end phalanx.

Etiopathogenesis
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Unsolved. Discussed are autosomal dominant inheritance, medication during pregnancy, atavism.

Localization
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Predominantly occurring on the index finger. Localization on other fingers is possible. Toes are rarely affected. Infestation on one or both sides is possible.

Clinical features
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Mostly microonychia or polyonychia, less frequently onychogrypose. Radiological narrowing of the distal phalanx, dorsal spur formation at the distal phalanx.

Therapy
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The disease is harmless; no causal therapy is known.

Literature
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  1. Bittar EO et al (1988) Congenital ischemic onychodystrophy (Iso-Kikuchi syndrome) and chronic lupus erythematosus. Dermatologist 39: 750-752
  2. Haneke E et al (1984) Congenital onychodysplasia: Iso-Kikuchi syndrome. dermatologist 35: 468-471
  3. Iso R (1969) Congenital nail defects of the index finger and reconstructive surgery. Seikei Geka 20: 1383-1384
  4. Kikuchi I, Horikawa S, Amano F (1974) Congenital onychodysplasia of the index fingers. Arch Dermatol 110: 743-746
  5. Thappa DM et al (2002) Polyonychia in congenital onychodysplasia of the index finger (Iso and Kikuchi syndrome): a report from India. J Dermatol 29: 603-605

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Last updated on: 27.11.2022