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Pachydermodactyly.

C M Reichert, J Costa, S H Barsky

    Clinical Orthopaedics and Related Research
    |April 1, 1985
    PubMed
    Summary
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    Pachydermodactyly is a rare fibromatosis causing finger swelling due to dense fibrosis around joints. Biopsies reveal benign fibroblasts and altered collagen, particularly increased Types III and V, in affected digits.

    Area of Science:

    • Dermatology
    • Pathology
    • Rheumatology

    Background:

    • Pachydermodactyly is a rare fibromatosis characterized by digital fibrotic proliferation.
    • It typically affects young adults, presenting as asymptomatic finger swelling.

    Observation:

    • The condition involves dense fibrosis and fibroblastic proliferation around proximal interphalangeal joints.
    • Affected areas include the dorsal and appositional aspects of digits II-V, sparing thumbs and lateral index/medial fifth digits.
    • Skin biopsies show marked dermal thickening, collagen extension into subcutaneous tissue, and poor dermal layer demarcation.

    Findings:

    • Fibroblast cytology in affected tissues is benign.
    • Collagen analysis reveals Types I, III, and V, with elevated Types III and V, consistent with fibromatoses.

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  • Electron microscopy indicates an increased number of fine-diameter collagen fibers compared to normal dermis.

    • Implications:

    • Understanding the specific collagen profile in pachydermodactyly aids in differentiating it from other fibrotic conditions.
    • These findings contribute to the histopathological characterization of pachydermodactyly.
    • Further research may elucidate the pathomechanisms driving altered collagen synthesis in this condition.