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

Terziroli Beretta-Piccoli, Benedetta; Guillod, Caroline; Marsteller, Igor; Blum, Roland; Mazzucchelli, Luca; Mondino, Chiara; Invernizzi, Pietro; Gershwin, M Eric; Mainetti, Carlo (2017). Primary Biliary Cholangitis Associated with Skin Disorders: A Case Report and Review of the Literature. Archivum immunologiae et therapiae experimentalis, 65(4), pp. 299-309. Springer 10.1007/s00005-016-0448-0

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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.

Item Type:

Journal Article (Review Article)


04 Faculty of Medicine > Department of Dermatology, Urology, Rheumatology, Nephrology, Osteoporosis (DURN) > Clinic of Dermatology

UniBE Contributor:

Blum, Roland


600 Technology > 610 Medicine & health








Sandra Nyffenegger

Date Deposited:

19 Mar 2018 10:25

Last Modified:

23 Oct 2019 08:02

Publisher DOI:


PubMed ID:


Uncontrolled Keywords:

Acquired reactive perforating dermatosis Case report Primary biliary cholangitis Review of the literature Skin disorders




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