Ainhum syndrome L94.6

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Dactylosis spontanea

Definition
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Predominantly occurring in Africans, independent, clinically and radiologically definite, aetiologically unexplained, circular laceration of distal extremities with subsequent spontaneous amputation; usually lasting for years.

Etiopathogenesis
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Chronic trauma and infections (barefoot walk) as well as reduced blood supply are discussed; questionable relation to keratosis palmoplantaris mutilans (see Pseudoainhum syndrome).

Manifestation
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For people living in tropical regions, especially middle-aged Africans.

Localization
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Mainly metatarsophalangeal joint, usually on both sides.

Clinical features
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Circular, continuously deepening laceration, edema of the distal phalanges, finally dry gangrene and spontaneous amputation. Mostly a course lasting for years.

Differential diagnosis
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Pseudoainhum syndrome (congenital!)

Therapy
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Textile clothing, early professional wound treatment, plastic surgery reconstruction if necessary.

Note(s)
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The term "Ainhum" comes from the African Yoruba dialect and means "saw, saw off".

Case report(s)
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A 42-year-old African woman complained of painful fissures of the small toes. Clinical presentation showed edematous swelling of both end phalanges proximally limited by deep constrictions with hyperkeratosis, rhagades and linear indurations. The right distal phalanx showed no bony continuity and could be easily moved to and fro by dangling. Therapy: Partial amputation of the right small toe.

Literature
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  1. Castori M et al (2010) Palmoplantar keratoderma, pseudo-ainhum, and universal atrichia: Anew patient and review of the palmoplantar keratoderma-congenital alopecia syndrome. At J Med Genet A 152A: 2043-2047.
  2. Roesch A et al (2007) A patient with dangling toe. JDDG 11: 1008-1009
  3. Shtofmakher G et al (2014) Autoamputation of the fifth digit: ainhum (dactylolysis spontanea). BMJ Case Rep doi: 10.1136/bcr-2014-205021.

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020