Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0032463 (polycythemia vera)
 3,374 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of pyoderma gangrenosum of the lip occurring in association with paroxysmal nocturnal haemoglobinuria is described. This is an extremely rare association, which has been documented in the literature on only two previous occasions. Pyoderma gangrenosum (PG) is an uncommon ulcerative skin disorder of unknown aetiology. Its clinical appearance is often distinctive, with established lesions consisting of a necrotic ulcer surrounded by a ragged undermined violaceous edge. Lesions are usually painful and are most often found on the lower limbs but can occur on the trunk, head and neck. The diagnosis is essentially clinical as there are no characteristic histopathological changes. Since its original description in 1930, PG has been frequently associated with a number of underlying systemic diseases. Foremost among these are inflammatory bowel disease and inflammatory polyarthritis. The association with haematological disorders is also well recognized, and includes acute and chronic lymphocytic and myeloid leukaemias, polycythaemia rubra vera, myelofibrosis, myelodysplastic syndrome, essential thrombocythaemia, hypogammaglobinaemia, monoclonal gammopathy, multiple myeloma and non-Hodgkin's lymphoma. We report a case of PG occurring on the lower lip of a 26-year-old man recently diagnosed as having paroxysmal nocturnal haemoglobinuria (PNH).
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PMID:Pyoderma gangrenosum associated with paroxysmal nocturnal haemoglobinuria. 803 97

Pyoderma gangrenosum (PG) is a rare, noninfectious neutrophilic dermatosis. Clinically, it begins with sterile pustules that rapidly progress into painful ulcers of variable depth and size with undermined violaceous borders. The diagnosis of PG is based on the history of an underlying disease, a typical clinical presentation, histopathology, and exclusion of other diseases. The peak incidence occurs between the ages of 20 to 50 years with women being more often affected than men. There have been very few reports of pyoderma gangrenosum with oral mucosal involvement. Oral lesions in previously reported cases have included ulcers of varying sizes from a few mm to several cm and have been reported to have been found on the tongue, soft and hard palate, buccal mucosa, and gingiva. Some of these oral lesions have been associated with ulcerative colitis, inflammatory bowel disease, and polycythemia rubra vera. A few cases were reported with biopsy findings, the histological picture being nonspecific, showing ulceration, and necrosis with inflammatory cell infiltrate. A peculiar case of pyoderma gangrenosum with an oral lesion is presented here, and the differential diagnosis is discussed.
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PMID:Pyoderma gangrenosum with oral involvement - case report and review of the literature. 2073 38

An association between hematological cancers and inflammatory bowel disease (IBD) has previously been suggested, but the risk of IBD in patients with myeloproliferative neoplasms (MPNs) is unknown. We conducted a nationwide population-based cohort study using Danish registries, to estimate the risk of IBD in individuals diagnosed with essential thrombocythemia, polycythemia vera, myelofibrosis or unclassifiable MPN during 1994-2013. MPN patients were matched 1:10 with sex- and age-matched comparisons. Everyone was followed until a diagnosis of IBD, death/emigration, or 31 December 2013. The risk of IBD overall and according to MPN subtype was calculated using Cox regression and presented as hazard ratios (HRs) with 95% confidence intervals (CI). Of 8207 MPN patients followed for 45,232 person-years, 80 were diagnosed with IBD (61 ulcerative colitis, 19 Crohn's disease). The rate of IBD per 1000 person-years was 1.8 (95% CI:1.4-2.2) in patients vs. 0.8 (95% CI:0.7-0.8) in comparisons, and the absolute 10-year risk of IBD was 0.8% (95% CI:0.6-1.0) in patients vs. 0.4% (95% CI:0.4-0.5) in comparisons. The HR of IBD was 2.4 (95% CI:2.1-2.9) with similar HRs for ulcerative colitis and Crohn's disease. MPN subtype risks varied from 2.1 (95% CI:1.6-2.7) to 2.8 (95% CI:2.1-3.7). Our unselected cohort study showed a more than 2-fold increased risk of IBD in MPN patients.
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PMID:Risk of Inflammatory Bowel Disease in Patients with Chronic Myeloproliferative Neoplasms: A Danish Nationwide Cohort Study. 3296 27