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pemfigus benígny familiárny

  1. Subject h.pemfigus benígny familiárny
    Subject h.Pemphigus, Benign Familial
    Entry termspemfigus benígny chronický
    pemfigus benígny familiárny chronický
    Haileyova-Haileyova choroba
    Hailey-Haileyova choroba
    English X referencesChronic Benign Familial Pemphigus
    Familial Benign Chronic Pemphigus
    Hailey-Hailey Disease
    Scope note in EnglishAn 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.
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