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[Linear Darier disease].

Silvestre Martínez1, Angel Vera, Carmen Eloy-García

  • 1Servicio de Dermatología y Venereología, Complejo Hospitalario Universitario Carlos Haya, Málaga, Spain. silvestremg@eresmas.net

Actas Dermo-Sifiliograficas
|April 6, 2006
PubMed
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Darier disease, a genetic skin disorder, can present in a rare linear pattern. This case highlights a 22-year-old woman diagnosed with linear Darier disease based on characteristic skin lesions and histology.

Area of Science:

  • Dermatology
  • Genetics
  • Histopathology

Background:

  • Darier disease is an autosomal dominant genodermatosis.
  • It can manifest in unusual linear or segmental patterns.
  • Early diagnosis is crucial for management.

Observation:

  • A 22-year-old woman presented with a 3-year history of small, linearly arranged, yellowish-brown papules on her right lower extremity.
  • No other skin, mucous membrane, or appendage involvement was noted.
  • The patient had no significant personal or family medical history.

Findings:

  • Histological examination revealed suprabasal acantholysis and numerous dyskeratotic cells.
  • These findings are characteristic of Darier disease.
  • The clinical presentation and histology confirmed a diagnosis of linear Darier disease.

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Implications:

  • This case expands the understanding of Darier disease's varied clinical presentations.
  • Recognizing linear Darier disease is important for accurate diagnosis and patient counseling.
  • Further research into genetic variations may elucidate the pathogenesis of segmental Darier disease.