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Pachydermodactyly in two young girls

F Bardazzi1, I Neri, P A Fanti

  • 1Department of Dermatology, University of Bologna, Italy.

Pediatric Dermatology
|July 1, 1996
PubMed
Summary

Pachydermodactyly, a rare fibromatosis, was observed in two girls with Ehlers-Danlos syndrome and tuberous sclerosis. These cases suggest systemic disease or obsessive-compulsive behavior may contribute to pachydermodactyly development.

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Area of Science:

  • Dermatology
  • Genetics
  • Pediatrics

Background:

  • Pachydermodactyly is a benign fibromatosis typically affecting young males.
  • Its occurrence in females, particularly those with underlying genetic conditions, is rare.

Observation:

  • This study details two pediatric cases of pachydermodactyly.
  • Case 1: A girl with Ehlers-Danlos syndrome (EDS).
  • Case 2: A girl with tuberous sclerosis.

Findings:

  • In the tuberous sclerosis patient, pachydermodactyly is hypothesized as a manifestation of the systemic disease.
  • In the Ehlers-Danlos syndrome patient, obsessive-compulsive behavior is proposed as the primary cause.
  • This highlights potential diverse etiologies for pachydermodactyly.

Implications:

  • Broadens understanding of pachydermodactyly's potential causes beyond typical demographics.
  • Suggests considering systemic conditions and behavioral factors in diagnosing pediatric fibromatosis.
  • Emphasizes the need for comprehensive evaluation in atypical presentations of pachydermodactyly.

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