Primary Biliary Cholangitis and Pruritus

Primary Biliary Cholangitis and Pruritis

This 57 yo woman was first seen in February 2018 for generalized pruritus.  She suffered with insomnia secondary to the symptoms. Her long-term medications include lithium, Effexor (venlafaxine) and Abilify (aripiprazole).   Because of abnormal LFTs she was referred to a gastroenterologist.  A liver biopsy “was suspicious for primary biliary cholangitis vs. primary sclerosing cholangitis.”  She was started on ursadiol 500 mg b.i.d. and cholestyramine 4 gm b.i.d. 

O/E: This woman appeared tired, depressed and older than stated age.  There were excoriated papules on her arms and legs and accessible areas of torso.

Clinical Photo:

Lab: Because of initial symptoms, CBC and Chem profile were ordered in February 2018

CBC normal

Initial Chen Profile: Alk phos 718 IU   (nl 18 – 218)

                                 SGOT 309            (nl 15 – 37)

                                 SGPT 446            (nl 15 – 56)

Repeat 10.18)          AlK Phos 319

                                SGOT  40

                                SGPT  57

                                Bilirubin has always been wnl.

Pathology: Biopsy of a papules on her right leg showed irregular epidermal hyperplasia, markedly dilated and disrupted follicles containing necrotic debris and a few PAS + spores. Gram stain negative.  DX: Perifollicular fibrosis suggestive of perforating folliculitis.

Comment:  In spite of normalization of LFTs on ursadiol and cholestyramine her pruritus did not improve.  The etiology of pruritus in PBC is unclear.  She has been started on Rifampin 150 mg b.i.d. and Narrow Band UVB.  I assume her skin lesions are secondary to PBC pruritus and excoriations. Your comments will be helpful.

Reference:
Excellent reference suggested by Dr. Richard Sontheimer:

1. A systematic approach to the management of cholestatic pruritus in primary biliary cirrhosis.  Hegade VS, wt. al.  Frontline Gastroenterol. 2016 Jul;7(3):158-166.   Free Full Text

Dr. Amanda Oakley suggested the leg lesions look like porokeratosis.  The association has been reported once in the literature.

2.Porokeratosis in primary biliary cirrhosis during plasmapheresis.  Venencie PY, Verola O, Puissant A. J Am Acad Dermatol. 1986 Oct;15(4 Pt 1):709-10.  Free Full Text.