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pemfigus benígny familiárny

  1. Heslopemfigus benígny familiárny
    Heslo anglickyPemphigus, Benign Familial
    Odkazypemfigus benígny chronický
    pemfigus benígny familiárny chronický
    Haileyova-Haileyova choroba
    Hailey-Haileyova choroba
    Angl. X odkazyChronic Benign Familial Pemphigus
    Familial Benign Chronic Pemphigus
    Hailey-Hailey Disease
    Vysvetľujúca pozn. v angl.An autosomal dominantly inherited skin disorder characterized by recurrent eruptions of vesicles and BULLAE mainly on the neck, axillae, and groin. Mutations in the ATP2C1 gene (encoding the secretory pathway Ca2++/Mn2++ ATPase 1 (SPCA1)) cause this disease. It is clinically and histologically similar to DARIER DISEASE - both have abnormal, unstable DESMOSOMES between KERATINOCYTES and defective CALCIUM-TRANSPORTING ATPASES. It is unrelated to PEMPHIGUS VULGARIS though it closely resembles that disease.
    Odkazy (8) - ČLÁNKY
    (1) - KNIHY
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Number of the records: 1  

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