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[Systemic mastocytosis and intestinal malabsorption].

H Barrière, B Dreno, C Pecquet

    La Semaine Des Hopitaux : Organe Fonde Par L'Association D'Enseignement Medical Des Hopitaux De Paris
    |November 17, 1983
    PubMed
    Summary
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    Systemic mastocytosis can cause intestinal malabsorption, characterized by steatorrhea and villous atrophy. Investigating mastocytosis is crucial for patients with malabsorption, especially those with specific skin lesion appearances.

    Area of Science:

    • Gastroenterology
    • Dermatology
    • Immunology

    Background:

    • Systemic mastocytosis is a rare disorder characterized by abnormal mast cell accumulation.
    • Intestinal malabsorption presents a diagnostic challenge, with various underlying causes.
    • Cutaneous manifestations are common in systemic mastocytosis.

    Observation:

    • This review examines 34 cases of systemic mastocytosis associated with intestinal malabsorption.
    • Analysis focused on characteristic intestinal anomalies and their correlation with the systemic condition.

    Findings:

    • Key intestinal findings include steatorrhea (fat malabsorption), partial villous atrophy, and secretory IgA deficiency.
    • Specific skin lesion characteristics, such as a telangiectatic and angiomatous appearance, may suggest this association.

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    Implications:

    • Routine mastocytosis screening is recommended for patients presenting with unexplained malabsorption.
    • Early diagnosis of systemic mastocytosis can guide appropriate management and improve patient outcomes.
    • Dermatological assessment may provide crucial clues for diagnosing gastrointestinal manifestations of mastocytosis.