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[Blue rubber bleb nevus (author's transl)].

P Laugier

    Annales De Dermatologie Et De Venereologie
    |August 1, 1978
    PubMed
    Summary

    Bean's syndrome (BRBN) is a rare hereditary condition characterized by bluish skin nevi and gastrointestinal angiomas, potentially causing severe bleeding. Treatment focuses on managing bleeding and anemia, with surgical removal of skin lesions being an option.

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    Area of Science:

    • Dermatology
    • Gastroenterology
    • Pathology

    Context:

    • Bean's syndrome (BRBN) is a rare hereditary disorder.
    • It presents with characteristic bluish skin nevi and gastrointestinal tract angiomas.
    • These angiomas can lead to significant bleeding complications.

    Purpose:

    • To describe the clinical and histological features of Bean's syndrome.
    • To highlight the association between skin and gastrointestinal angiomas.
    • To discuss the diagnostic and prognostic implications.

    Summary:

    • Bean's syndrome involves hereditary dysembryoplasia with bluish skin nevi and gastrointestinal angiomas.
    • Skin angiomas manifest in various forms, including cavernous, nipple-like, or irregular blue marks.
    • Gastrointestinal angiomas, located in the submucosa, can cause hematemesis and melena, similar to Rendu-Osler disease.

    Impact:

    • Understanding Bean's syndrome is crucial for diagnosing and managing patients with unexplained bleeding.
    • The prognosis is primarily determined by the severity of bleeding and associated anemia.
    • While skin angiomas can be surgically excised, gastrointestinal lesions require careful management to prevent complications.

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