Primary Biliary Cholangitis Associated with Skin D

Authors: Benedetta Terziroli BerettaPiccoli Caroline Guillod Igor Marsteller Roland Blum Luca Mazzucchelli Chiara Mondino Pietro Invernizzi M Eric Gershwin Carlo Mainetti

Publish Date: 2017/01/25

Volume: 65, Issue: 4, Pages: 299-309

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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 immunemediated 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 highquality data on skin conditions associated with PBC

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