Systemic lupus erythematosus complicated with liver cirrhosis in a patient with Papillon-Lefèvre syndrome

H Yasudo; T Ando; M Takeuchi; H Nakano; T Itonaga; H Takehara; T Isojima; K Miura; Y Harita; J Takita; A Oka

doi:10.1177/0961203314547794

Systemic lupus erythematosus complicated with liver cirrhosis in a patient with Papillon-Lefèvre syndrome

Lupus. 2014 Dec;23(14):1523-7. doi: 10.1177/0961203314547794. Epub 2014 Aug 14.

Authors

H Yasudo¹, T Ando², M Takeuchi³, H Nakano⁴, T Itonaga⁵, H Takehara⁵, T Isojima⁵, K Miura⁵, Y Harita⁵, J Takita⁵, A Oka⁵

Affiliations

¹ Department of Pediatrics, Tokyo University Graduate School of Medicine, Tokyo, Japan [email protected].
² Department of Pediatrics, Tokyo University Graduate School of Medicine, Tokyo, Japan Laboratory for Allergic Disease, RIKEN Center for Integrative Medical Sciences, Yokohama, Japan.
³ Kikkoman General Hospital, Chiba, Japan.
⁴ Department of Dermatology, Hirosaki University Graduate School of Medicine, Aomori, Japan.
⁵ Department of Pediatrics, Tokyo University Graduate School of Medicine, Tokyo, Japan.

PMID: 25124675
DOI: 10.1177/0961203314547794

Abstract

We report the first case of a girl who presented with Papillon-Lefèvre syndrome (PLS) and subsequently developed systemic lupus erythematosus and liver cirrhosis. This indicates that autoimmune diseases can be a complication in patients with PLS. Cathepsin C gene mutations were not found in our patient or her mother. Thus, other genetic factors may have been involved in this patient.

Keywords: Cirrhosis; Papillon-Lefèvre syndrome; systemic lupus erythematosus.

Publication types

Case Reports

MeSH terms

Child
Female
Hepatitis, Autoimmune / complications
Humans
Liver Cirrhosis / etiology*
Liver Cirrhosis / pathology
Lupus Erythematosus, Systemic / etiology*
Papillon-Lefevre Disease / complications*
Papillon-Lefevre Disease / genetics