Background: Pachydermodactyly is an infrequently reported disease characterized clinically by painless swelling of the soft tissues at interphalangeal joints. Histopathologic features include increased dermal accumulation of collagen fibers. Young males are predominantly affected. There are only approximately 50 cumulative cases reported worldwide.
Objective: : This is a review of 6 new cases of pachydermodactyly from our center.
Methods: Characteristics of our cases are described in detail and the literature of this disease is reviewed.
Results: The mean disease onset age of our 6 patients was 14 +/- 2.7 years; 5 of the patients were male and 1 was female. Clinical presentations included chronic noninflammatory bulbous swelling of proximal interphalangeal (PIP) joints. Mean numbers of involved joints were 5 +/- 3; most frequently affected were PIP2, 3, and 4 symmetrically. There was 1 patient with monoarticular involvement. X-ray demonstrated soft tissue swelling with no bony or articular damage. Histopathology displayed increased dermal deposition of collagen fibers.
Conclusions: Pachydermodactyly is not well recognized by rheumatologists and should be differentiated from inflammatory arthritis involving PIP joints.
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