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.

Friday, November 02, 2018

Parameatal Cyst

Presented by:
Caitlin Hogue, MBChB
The Launceston Skin Centere
Launceston, Tasmania

A 10 year old boy presented to our clinic with a two month history of an asymptomatic lesion adjacent to the urethral meatus.

O/E:  There is a 4 mm in diameter cystic lesion in the above-described area.

Clinical Photo:

 Diagnosis:  Parameatal Cyst

Plan: This is an unusual lesion.  Although the definitive treatment is surgical excisison, a small lesion may be observed.  Little is known about the behaviour of these uncommonly observed lesions.  We will try to identify a paediatric urologist who has some expertise with these lesions.

Reference: Parameatal Cyst: A Presentation of Rare Case and Review of Literature. Lal S and Agarwal Ankur. J Clin Diagn Res. 2013 Aug; 7(8): 1757–1758.  Free Full Text.
Abstract: A parameatal urethral cyst is a very rare congenital anomaly. It was first reported in two males in 1956 by Thompson and Lantin. About 50 cases have been published since then. Most of the cases which have been reported were from Japanese population and on extensive literature search, few cases were found to have been reported from India. We are reporting a case of a parameatal urethral cyst in a 7-year-old boy. Complete excision of the cyst with total removal of the epithelium is required for treatment and for prevention of recurrence.