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A case of deforming pachydermodactyly.
Regina Taylor-Gjevre1, Anurag Saxena, Sharif El Maadawy
1Department of Medicine, University of Saskatchewan, Saskatoon, Canada. r.gjevre@usask.ca
Summary
Pachydermodactyly, a rare fibromatosis, caused progressive digital deformities in a patient. Increased collagen production led to joint instability and functional impairment, differing from previous reports.
Area of Science:
- Dermatology
- Orthopedics
- Pathology
Background:
- Pachydermodactyly is a rare benign fibromatosis affecting the proximal interphalangeal joints (PIPs).
- It typically presents as soft tissue swelling and digital deformities.
Observation:
- A 39-year-old man presented with progressive digital deformation and flexion deformities at the PIPs.
- Radiographs showed nonerosive subluxation and significant soft tissue swelling.
Findings:
- Biopsy confirmed increased collagen production and fibroblast/myofibroblast proliferation, consistent with pachydermodactyly.
- Extensive soft tissue involvement altered articular supporting structures, leading to deformity and functional impairment.
Implications:
- This case highlights a severe presentation of pachydermodactyly with significant functional consequences.
- Understanding the extent of soft tissue involvement is crucial for managing this rare condition.
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