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[Costa acro-kerato-elastoidosis]

O Boudghène-Stambouli1, A Merad-Boudia, M R Benkalfat

  • 1Service de Dermato-Vénéréologie, Centre Hospitalo-Universitaire, Tlemcen, Algérie.

Annales De Dermatologie Et De Venereologie
|January 1, 1993
PubMed
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Acrokeratoelastoidosis of Costa, a rare skin condition, presented in two sisters. This report details their unique presentation and suggests a possible autosomal recessive inheritance pattern, differing from typical findings.

Area of Science:

  • Dermatology
  • Genetics
  • Histopathology

Background:

  • Acrokeratoelastoidosis of Costa is a rare genodermatosis.
  • Typically, it exhibits dominant or sporadic inheritance patterns.
  • This study investigates a familial case with a potentially different transmission mode.

Observation:

  • Two sisters presented with lenticular papules on hands and feet.
  • Lesions varied from skin-colored papules to translucent, keratotic plaques.
  • Histopathology revealed hyperkeratosis, acanthosis, and a diminished elastic network in the dermis.

Findings:

  • The patients displayed typical acrokeratoelastoidosis lesions.
  • A potential autosomal recessive inheritance pattern was observed in this familial case.

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  • This contrasts with the commonly reported dominant or sporadic inheritance.
  • Implications:

    • This case expands the understanding of acrokeratoelastoidosis of Costa's clinical spectrum.
    • The potential autosomal recessive inheritance warrants further investigation in genetic studies.
    • Further research is needed to clarify the genetic basis and inheritance patterns of this condition.