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[Pseudoainhum in lamellar ichthyosis].

C Dornier1, J F Cuny, M P Di Cesare

  • 1Service de Dermatologie, CHR Metz Thionville, Hôpital Beauregard, Thionville.

Annales De Dermatologie Et De Venereologie
|March 23, 2002
PubMed
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Pseudoainhum, a condition causing constricting bands and potential digit amputation, is rarely seen with lamellar ichthyosis. This case highlights a new association between this rare dermatosis and keratinization disorders.

Area of Science:

  • Dermatology
  • Genetics
  • Pathology

Background:

  • Pseudoainhum is a rare condition characterized by constricting bands on digits, potentially leading to spontaneous amputation.
  • Etiologies are diverse, encompassing congenital factors and associated diseases.
  • Lamellar ichthyosis is a severe congenital ichthyosis characterized by generalized scaling and keratinization abnormalities.

Observation:

  • A case of a 30-year-old woman with severe lamellar ichthyosis who developed pseudoainhum on her third left finger.
  • Clinical presentation included a constricting band, painful evolution, and distal bone resorption on X-ray.
  • Standard biological tests were within normal limits.

Findings:

  • The patient's pseudoainhum was associated with severe lamellar ichthyosis, a previously undescribed comorbidity.

Related Experiment Videos

  • Radiographic findings revealed distal bone resorption in the affected phalanx.
  • Amputation of the distal phalanx was necessitated due to the painful progression and lack of conservative treatment options.

    • Implications:

    • This case suggests a potential link between pseudoainhum and the keratinization disorders characteristic of lamellar ichthyosis.
    • It expands the known spectrum of dermatoses associated with pseudoainhum.
    • Further research may elucidate the specific mechanisms underlying this association and inform management strategies for patients with both conditions.