Patients with pyoderma gangrenosum – analyses of the German DRG data from 2012

Abstract

Pyoderma gangrenosum (PG) is a rarely diagnosed non-infectious neutrophil ulcerative dermatosis with only limited knowledge on the underlying auto-inflammatory process. To unravel common cofactors and comorbidities in patients with PG we analysed Diagnosis Related Groups (DRG) cases of all inpatients diagnosed with PG in German hospitals in 2012. We received data of 1227 inpatient cases having PG as primary diagnosis and 985 inpatient cases with PG as secondary diagnosis. The ratio of women to men was 2:1, and the most often registered age was 75-79 years. Common comorbidities were arterial hypertension (50·3%), non-insulin-dependent diabetes mellitus (25·1%) and dysfunction of lipid metabolism (10·8%). In sum, 94·8% of the patients suffered from aspects of metabolic syndrome. Other comorbidities were Crohn's disease (4·5%), ulcerative colitis (4·2%), chronic polyarthritis (5·2%), monoclonal gammopathy or myelodysplastic syndrome (2·5%), leukaemia (1·1%) and lymphoma (0·4%). DRG data do not reflect individual patients, but rather patient cases. We described the worldwide largest PG population and confirmed a wide range of potentially relevant and partly not yet described cofactors and comorbidities such as metabolic syndrome.

Epidemiology of pyoderma gangrenosum: Results from an Italian prospective multicentre study

“…Actual diagnoses, delayed on average by 10 months, included vascular disease (occlusive or venous insufficiency), vasculitis, malignancies, infections, drug‐induced or exogenous tissue injury, and manifestations of other autoimmune diseases . In the DRG investigated by Jockenhöfer et al, 985 patients had a secondary diagnosis of PG, while the first was chronic leg ulcers in 75% of cases . The need to rule out an alternative disease should override the fear of exacerbating the condition (because of pathergy) by performing a biopsy .…”

“…Thirdly, 4 subjects (6.2%) had a haematological condition, and 11 (17.2%) had other underlying diseases, usually not described as associated with PG, such as arterial hypertension or diabetes mellitus type II. Nevertheless, in the retrospective multicentre study of Al Ghazal et al, a description is given about a significant association between PG and metabolic/endocrinological diseases, confirmed subsequently by the work on DRG data by Jockenhöfer et al, who report a prevalence of diabetes mellitus type II in 25% of patients affected by PG (>20% respect to normal adult German population) …”

“…PG is associated with underlying conditions in 50% to 75% of patients, and the most frequent association is with IBD (34%‐65.2%) and less frequently with arthritis (16.1%‐37%) and haematological disorders (6.5%‐20%) . In accordance with these data, we found an associated disease in 59.4% of cases, with IBD being the most common, affecting up to 16 patients (25%), even if less frequently than in literature.…”

“…analysed the incidence, mortality, and disease associations in the United Kingdom, reporting an incidence of between 6.9 and 9.1 cases per million population per year . Jockenhöfer et al, looking at the Diagnosis‐Related Group (DRG) introduced in Germany in 2005, reported 1227 patients with PG as the primary diagnosis and 985 patients with PG as secondary diagnosis in 2012 . Sadly, the main limitation of the DRG analysis is that it cannot permit the calculation of a real incident of PG.…”

Pioderma Gangrenoso Associado a Hemoglobinúria Paroxística Noturna

“…2 Existem relatos na literatura associando o PG ao fenómeno de patergia, ou seja, o surgimento de novas lesões desencadeado por traumas, incluindo cirúrgicos. 1,3,4 Acredita-se que o esclarecimento sobre a etiologia do PG esteja presente na associação com doenças sistêmicas (doenças reumatológicas, doenças inflamatórias intestinais, neoplasias malignas, doenças hematológicas dentre elas a hemoglobinúria paroxística noturna), [5][6][7][8] uma vez que tal relação está presente em 50 a 70% dos casos. 2,7 O PG também pode ser considerado idiopático, apresentando-se como uma lesão primária, isolada e restrita à pele.…”