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Bilateral destructive synovitis associated with alpha mannosidase deficiency.

S W Weiss, W D Kelly

    The American Journal of Surgical Pathology
    |July 1, 1983
    PubMed
    Summary

    A rare complication of alpha mannosidase deficiency (mannosidosis) was observed in a 13-year-old female, presenting as destructive ankle synovitis. This finding expands the known clinical spectrum of this lysosomal storage disorder.

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    Area of Science:

    • Biochemistry
    • Genetics
    • Histopathology

    Background:

    • Alpha mannosidase deficiency (mannosidosis) is a rare lysosomal storage disorder.
    • It is characterized by the accumulation of oligosaccharides in various tissues.
    • Clinical manifestations typically involve neurological and immunological dysfunction.

    Observation:

    • A 13-year-old female with biochemically confirmed mannosidosis presented with bilateral ankle synovitis.
    • The condition was initially misdiagnosed as pigmented villonodular synovitis.
    • Histological examination revealed characteristic cellular infiltration and material consistent with oligosaccharide accumulation.

    Findings:

    • The synovitis demonstrated bilateral symmetry and a monomorphic cell population.
    • Histochemical and ultrastructural analysis confirmed the presence of oligosaccharides within the synovium.

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  • This represents a previously unreported complication of alpha mannosidase deficiency.

    • Implications:

    • This case expands the recognized clinical spectrum of mannosidosis.
    • It highlights the importance of considering metabolic disorders in cases of unexplained synovitis.
    • Further research may elucidate the pathogenesis of this specific complication.