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

被引:13
|
作者
Beretta-Piccoli, Benedetta Terziroli [1 ]
Guillod, Caroline [2 ]
Marsteller, Igor [3 ]
Blum, Roland [4 ]
Mazzucchelli, Luca [5 ]
Mondino, Chiara [2 ]
Invernizzi, Pietro [6 ]
Gershwin, M. Eric [7 ]
Mainetti, Carlo [2 ]
机构
[1] Epatoctr Ticino, Via Soldino 5, Lugano, Switzerland
[2] Osped Reg Bellinzona, Dermatol Ente Osped Cantonale, Bellinzona, Switzerland
[3] Osped Reg Bellinzona, Serv Gastroenterol, Bellinzona, Switzerland
[4] Univ Bern, Dermatol Klin, Dermatopathol Labor, Bern, Switzerland
[5] Ist Cantonale Patol, Locarno, Switzerland
[6] Univ Milano Bicocca, San Gerardo Hosp, Program Autoimmune Liver Dis, Int Ctr Digest Hlth, Monza, Italy
[7] Univ Calif Davis, Sch Med, Div Rheumatol Allergy & Clin Immunol, 451 Hlth Sci Dr,Suite 6510, Davis, CA 95616 USA
关键词
Primary biliary cholangitis; Acquired reactive perforating dermatosis; Skin disorders; Case report; Review of the literature; PRIMARY SCLEROSING CHOLANGITIS; CIRRHOSIS OVERLAP SYNDROME; AUTOIMMUNE LIVER-DISEASE; T REGULATORY CELLS; ORAL LICHEN-PLANUS; CHANGING NOMENCLATURE; GENERALIZED MORPHEA; PERFORATING DERMATOSIS; SINGLE PATIENT; ET-ATROPHICUS;
D O I
10.1007/s00005-016-0448-0
中图分类号
R392 [医学免疫学]; Q939.91 [免疫学];
学科分类号
100102 ;
摘要
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.
引用
收藏
页码:299 / 309
页数:11
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