Primary biliary cirrhosis and the CREST syndrome: a report of 22 cases.
The syndrome of primary biliary cirrhosis and limited scleroderma (the CREST syndrome) is described in 22 patients. The syndrome is expanded to include a serologic marker--the anticentromere antibody--and keratoconjunctivitis sicca. The acronym 'PACK' (primary biliary cirrhosis, anticentromere antibody, CREST syndrome, and keratoconjunctivitis sicca) with 'syndrome' is suggested to facilitate recall of the various components of this entity.
Department of Dermatology, Mayo Clinic, Rochester, Minnesota 55905.
F C
FC
Powell
A L
AL
Schroeter
E R
ER
Dickson
D000328:Adult; D000368:Aged; D002114:Calcinosis / Q000473:pathology; D004935:Esophageal Diseases; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000473:pathology; D008875:Middle Aged; D011928:Raynaud Disease / Q000473:pathology; D012595:Scleroderma, Systemic / Q000473:pathology; D012871:Skin Diseases / Q000473:pathology; D013577:Syndrome; D013684:Telangiectasis