MeSH 搜索器

Pemphigus, Benign Familial

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.
推出的年份: 1992
副标题
树号: C16.320.850.700, C17.800.827.700, C17.800.865.858
MeSH 单一 ID: D016506
进入的组:
  • Benign Familial Pemphigus
  • Familial Pemphigus, Benign
  • Chronic Benign Familial Pemphigus
  • Benign Chronic Pemphigus
  • Familial Benign Chronic Pemphigus
  • Hailey-Hailey Disease
  • Hailey Hailey Disease
早前的内容:
  • Pemphigus (1966-1991)

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