Tuesday, November 27, 2018

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.

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.


  1. Dr. Warren Heymann wrote: "“Tough case. There has been increasing literature about neurokinin inhibitors such as aprepitant. It may be worth a try should there be no response to NBUVB."

  2. Khalifa Sharquie from Baghdad, Iraq wrote: "This is not the typical skin manifestation of primary biliary cirrhosis and the present rash is that of prurigo nodularis which is related to her emotional upset.Tofranil tab with strong topical steroid would be very helpful,in addition to her psychological support."

  3. Dr. Bhushan KumarNovember 28, 2018

    some of the skin lesions look like subcorneal pustular dermatosis(IgA disease). For itching some people respond better to Doxepin.

  4. The images in my opinion show a primary skin disease rather than simple excoriations. Each plaque is similar to the other and has an elevated rim. Could she have 2 problems: pruritus due to PBC and disseminated superficial actinic porokeratosis (you could ask the pathologist to look for cornoid lamella if this was biopsied, otherwise repeat biopsy of the elevated rim of a representative lesion)

  5. Comments of Arthur Wasser (Gastroenterologist): This lady with with an ill defined cholestatic disease (possibly even PBC thogh not supported by the biopsy) is typical in the unremitting pruritis of cholestatic liver disease. Treatment is ofte frustrating and what I find interesting is that the history does not go on to show whether the addition of Rifampicin was helpful in relieving her pruritis. The cause of the pruritis remains unknown to this day and the mechanism of action of the Rifampin likewise is unknown. Nodular pruritic lesions have been reported, but I have never seen them.

  6. from Dr. Dato Cheng Leng, Malaysia:

    12:03 PM (3 minutes ago)

    to me
    Dear Dr Elpern,

    Thanks for the email. I’m yet to work out how to join the blog directly.

    I have a few contributions towards this patient:-

    1. I think she has both PBC and disseminated superficial actinic porokeratosis, kobnerisation may be involved in the evolution from one to another. Her pruritus is hence complex, we need to break it down into its components to deal with individually yet simultaneously, to achieve the so called synergistic effects:-
    a) continue to find the best treatment for her PBC;
    b) avoid sunlight and use appropriate sunscreens.
    c) try Ingenol mebutate gel for the porokeratosis lesions.
    Topical treatment is always preferred with her biliary pathologies and in accordance to basic principles of skin therapies.
    d) do not forget to try the longest and quite innocuous age-old oral chlorpheramine,
    this and the topical gels, plus the very safe atopiclare lotion may just break her itch scratch vicious cycle!

    2) her ruptured follicles are just due to the trauma of intensive excoriation.

    3) pray for better control of a difficult itch....

    Thank you.


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