title: High frequency of cholestasis in generalized pustular psoriasis: Evidence for neutrophilic involvement of the biliary tract.
abstract: Generalized pustular psoriasis is a rare form of psoriasis that is sometimes associated with extracutaneous manifestations. Evidence for biliary involvement has been suggested in isolated cases. We investigated the prevalence and nature of liver abnormalities occurring in this disease. Twenty-two patients consecutively admitted for generalized pustular psoriasis who underwent liver biological tests at the time of the attack and during the following weeks were included. Twenty patients (90%) had at least one abnormal biological liver parameter. Eleven patients (50%) had pronounced abnormalities: jaundice (4/22), gammaglutamyl transferase higher than 5 times the normal value (10/22), alkaline phosphatase higher than twice the normal value (7/22), and/or aminotransferases higher than 3 times the normal value (7/22). These abnormalities returned to normal range at the time of remission of pustular psoriasis, suggesting that severe liver abnormalities could be associated with severe cutaneous disease. Neutrophilic cholangitis was observed on liver biopsy. Persistent magnetic resonance cholangiopancreatography features similar to those observed in sclerosing cholangitis were found in 3 of the 4 patients studied. No causal factor other than pustular psoriasis could be identified. In conclusion, our results demonstrate the high frequency of liver abnormalities in patients with generalized pustular psoriasis. Biliary involvement related to neutrophilic cholangitis should be added to the spectrum of extracutaneous manifestations of this disease, and physicians should be aware of such a complication.
authors.affiliation: Department of Dermatology 1, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris and Université Paris 7, Paris, France. mviguier@libertysurf.fr
authors.forename: Manuelle
authors.initials: M
authors.lastname: Viguier
authors.forename: Matthieu
authors.initials: M
authors.lastname: Allez
authors.forename: Anne-Marie
authors.initials: AM
authors.lastname: Zagdanski
authors.forename: Philippe
authors.initials: P
authors.lastname: Bertheau
authors.forename: Eric
authors.initials: E
authors.lastname: de Kerviler
authors.forename: Michel
authors.initials: M
authors.lastname: Rybojad
authors.forename: Patrice
authors.initials: P
authors.lastname: Morel
authors.forename: Louis
authors.initials: L
authors.lastname: Dubertret
authors.forename: Marc
authors.initials: M
authors.lastname: Lémann
authors.forename: Hervé
authors.initials: H
authors.lastname: Bachelez
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000469:Alkaline Phosphatase / Q000097:blood; D001659:Biliary Tract / Q000473:pathology*; D001660:Biliary Tract Diseases / Q000000981:diagnostic imaging / Q000209:etiology / Q000473:pathology; D002760:Cholangiopancreatography, Endoscopic Retrograde; D002761:Cholangitis / Q000150:complications / Q000473:pathology; D002779:Cholestasis / Q000175:diagnosis / Q000453:epidemiology* / Q000209:etiology*; D005260:Female; D006801:Humans; D007565:Jaundice / Q000209:etiology; D008099:Liver / Q000000981:diagnostic imaging / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008499:Medical Records; D008875:Middle Aged; D009504:Neutrophils / Q000473:pathology*; D010297:Paris / Q000453:epidemiology; D015995:Prevalence; D011565:Psoriasis / Q000150:complications* / Q000201:enzymology; D012720:Severity of Illness Index; D000637:Transaminases / Q000097:blood; D005723:gamma-Glutamyltransferase / Q000097:blood
chemical_list: D005723:gamma-Glutamyltransferase; D000637:Transaminases; D000469:Alkaline Phosphatase