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Primary sclerosing cholangitis associated with pyostomatitis vegetans and pyodermatitis vegetans: A potentially bilious situation

DII small banner By Warren R. Heymann, MD
Aug. 29, 2016

Inflammatory bowel disease may be associated with multiple cutaneous manifestations including pyoderma gangrenosum (PG), erythema nodosum, or directly by fistulae or “metastatic” Crohn disease. Seven cases of pyostomatitis vegetans-pyodermatitis vegetans (PSV-PDV) were reviewed. All 7 patients had inflammatory bowel disease (IBD) — 3 had Crohn disease (CD) and 4 ulcerative colitis (UC). Two patients manifested recalcitrant PG. Three cases had primary sclerosing cholangitis (PSC). Of those with PSC, 1 was associated with CD and 2 with UC (1).

The association of PSV-PVD with IBD is well known. I was surprised to learn that the association of PSC with PSV-PVD was previously unknown, given the well-known association of IBD with PSC.

PSC is a chronic progressive disease of unknown etiology that results in strictures of the intra- and extrahepatic bile ducts. The majority of patients ultimately develop hepatic failure due to cirrhosis, requiring liver transplantation. The diagnosis of PSC is established once secondary causes of sclerosing cholangitis are rule out, such as gallstones, surgical trauma, AIDS, parasites, and the recently described IgG4-related sclerosing cholangitis (2). Importantly, prolonged duration of IBD is associated with an increased risk of cholangiocarcinoma in patients with PSC-IBD, and colectomy itself does not modify this risk. These findings identify a subset of patients who are at high risk of this lethal complication and in need of close surveillance (3).

As Clark et al have observed, PSV-PDV may precede gastroenterological symptoms. (1). Most dermatologists are probably cognizant of the association of PSV-PVD with IBD. The novel finding that almost half of the cases of PSV-PVD in this series were associated with PSC, and that PSC is associated with cholangiocarcioma, mandates that dermatologists recognize PSV-PDV as soon as possible, and make sure we refer to our GI colleagues posthaste.

1. Clark LG, et al: Pyostomatitis vegetans (PSV)-pyodermatits vegetans (PDV): A clinicopathologic study of 7 cases at a tertiary referral center. J Am Acad Dermatol 2016; 75: 578-84.
2. Moon SH, et al. Development of a scoring system for differentiating IgG4-related sclerosing cholangitis from primary sclerosing cholangitis. J Gastroenterol 2016 Jul 28 {Epub ahead of print]
3. Gulamhusein AF, et al. Duration of inflammatory bowel disease is associated with increased risk of cholangiocarcinoma in patients with primary sclerosing cholangitis and IBD. Am J Gastroenterol 2016; 111: 705-11.

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