title: Primary Biliary Cholangitis Associated with Skin Disorders: A Case Report and Review of the Literature.
abstract: Primary biliary cholangitis (PBC) is a rare autoimmune cholestatic liver disease. It is often associated with extrahepatic autoimmune diseases. Skin disorders are sporadically reported in association with PBC. We report an unusual case of PBC associated with acquired reactive perforating dermatosis (ARPD) and present a review of the literature on skin disorders associated with PBC. Our patient presented to the dermatology department with generalized pruritus associated with nodular perforating skin lesions on the trunk, and cholestatic liver disease of unknown origin. After having established both diagnosis of ARPD and PBC, she was managed in an interdisciplinary manner, and both her skin and liver conditions improved gradually. Only one similar case is reported in the literature, in that case, the liver disease was not treated. By reviewing the literature, we found that lichen planus, vitiligo, and psoriasis are the most frequent skin disorders associated with PBC. However, there is only limited data about specific skin disorders associated with PBC. This case report of a patient with PBC associated with ARPD underlines the importance of interdisciplinary management of patients with rare liver diseases combined with rare skin disorders. The present review of the literature shows that probably, immune-mediated skin conditions are not more frequent in PBC patients than in the general population. However, the available data are scant; there is a need for high-quality data on skin conditions associated with PBC.
authors.affiliation: Epatocentro Ticino, Epatocentro Ticino, Via Soldino 5, Lugano, Switzerland. benedetta.terziroli@hin.ch.
authors.forename: Benedetta
authors.initials: B
authors.lastname: Terziroli Beretta-Piccoli
authors.affiliation: Dermatologia Ente Ospedaliero Cantonale, Ospedale Regionale Bellinzona, Bellinzona, Switzerland.
authors.forename: Caroline
authors.initials: C
authors.lastname: Guillod
authors.affiliation: Servizio di Gastroenterologia Ospedale Regionale Bellinzona, Bellinzona, Switzerland.
authors.forename: Igor
authors.initials: I
authors.lastname: Marsteller
authors.affiliation: Dermatopathologie Labor, Dermatologische Klinik, Universität Bern, Bern, Switzerland.
authors.forename: Roland
authors.initials: R
authors.lastname: Blum
authors.affiliation: Istututo Cantonale di Patologia, Locarno, Switzerland.
authors.forename: Luca
authors.initials: L
authors.lastname: Mazzucchelli
authors.affiliation: Dermatologia Ente Ospedaliero Cantonale, Ospedale Regionale Bellinzona, Bellinzona, Switzerland.
authors.forename: Chiara
authors.initials: C
authors.lastname: Mondino
authors.affiliation: Program for Autoimmune Liver Diseases, International Center for Digestive Health, University of Milan-Bicocca, San Gerardo Hospital, Monza, Italy.
authors.forename: Pietro
authors.initials: P
authors.lastname: Invernizzi
authors.affiliation: Division of Rheumatology, Allergy and Clinical Immunology, University of California at Davis School of Medicine, 451 Health Sciences Drive, Suite 6510, Davis, CA, 95616, USA. megershwin@ucdavis.edu.
authors.forename: M Eric
authors.initials: ME
authors.lastname: Gershwin
authors.affiliation: Dermatologia Ente Ospedaliero Cantonale, Ospedale Regionale Bellinzona, Bellinzona, Switzerland.
authors.forename: Carlo
authors.initials: C
authors.lastname: Mainetti
mesh_terms: D000974:Antibodies, Antinuclear / Q000097:blood; D001327:Autoimmune Diseases / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology; D003872:Dermatitis / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology; D005260:Female; D006801:Humans; D008010:Lichen Planus / Q000453:epidemiology*; D008099:Liver / Q000276:immunology* / Q000473:pathology; D008105:Liver Cirrhosis, Biliary / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology; D008875:Middle Aged; D010348:Patient Care Team; D011565:Psoriasis / Q000453:epidemiology*; D012867:Skin / Q000276:immunology* / Q000473:pathology
chemical_list: D000974:Antibodies, Antinuclear
keywords: Acquired reactive perforating dermatosis; Case report; Primary biliary cholangitis; Review of the literature; Skin disorders