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Primary Biliary Cholangitis Associated with Skin Disorders: A Case Report and Review of the Literature

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Archivum Immunologiae et Therapiae Experimentalis Aims and scope

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.

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Abbreviations

PBC:

Primary Biliary Cholangitis

ARPD:

Acquired reactive perforating dermatosis

AMA:

Anti-mitochondrial antibodies

ANA:

Anti-nuclear antibodies

UDCA:

Ursodeoxycholic acid

BMI:

Body mass index

ULN:

Upper limit of norm

IQR:

Interquartile range

ELISA:

Enzyme-linked immunosorbent assay

SSY:

Sjögren’s syndrome

AIH:

Autoimmune hepatitis

SS:

Systemic sclerosis

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Authors and Affiliations

  1. Epatocentro Ticino, Epatocentro Ticino, Via Soldino 5, Lugano, Switzerland

    Benedetta Terziroli Beretta-Piccoli

  2. Dermatologia Ente Ospedaliero Cantonale, Ospedale Regionale Bellinzona, Bellinzona, Switzerland

    Caroline Guillod, Chiara Mondino & Carlo Mainetti

  3. Servizio di Gastroenterologia Ospedale Regionale Bellinzona, Bellinzona, Switzerland

    Igor Marsteller

  4. Dermatopathologie Labor, Dermatologische Klinik, Universität Bern, Bern, Switzerland

    Roland Blum

  5. Istututo Cantonale di Patologia, Locarno, Switzerland

    Luca Mazzucchelli

  6. Program for Autoimmune Liver Diseases, International Center for Digestive Health, University of Milan-Bicocca, San Gerardo Hospital, Monza, Italy

    Pietro Invernizzi

  7. 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

    M. Eric Gershwin

Authors
  1. Benedetta Terziroli Beretta-Piccoli
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  2. Caroline Guillod
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  3. Igor Marsteller
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  4. Roland Blum
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  5. Luca Mazzucchelli
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  6. Chiara Mondino
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  7. Pietro Invernizzi
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  8. M. Eric Gershwin
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  9. Carlo Mainetti
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Corresponding authors

Correspondence to Benedetta Terziroli Beretta-Piccoli or M. Eric Gershwin.

Additional information

Lab work performed at Ospedale Regionale Bellinzona, Switzerland.

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Terziroli Beretta-Piccoli, B., Guillod, C., Marsteller, I. et al. Primary Biliary Cholangitis Associated with Skin Disorders: A Case Report and Review of the Literature. Arch. Immunol. Ther. Exp. 65, 299–309 (2017). https://doi.org/10.1007/s00005-016-0448-0

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  • DOI: https://doi.org/10.1007/s00005-016-0448-0

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