Analysis of the German <scp>DRG</scp> data for livedoid vasculopathy and calciphylaxis

Renner, Regina; Dissemond, Joachim; Goerge, Tobias; Hoff, Norman-Philipp; Kröger, Knut; Erfurt‐Berge, Cornelia

doi:10.1111/jdv.14190

Cited by 17 publications

(14 citation statements)

References 34 publications

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“…The median age of the study population was 53 years (IQR: 40.5-68). Both facts are similar to data published by Renner et al 15 while others had described a female-to-male ratio of 3 : 1 16,17 and a clearly younger population with a mean age of 32 or 37 years. 18,19 The average BMI in our study population was only slightly lower (25.6 kg/m²) compared to an age-matched German population with a BMI of 26.4 kg/m² 20 although BMI was elevated in 40% of LV patients.…”

Section: Discussionsupporting

confidence: 92%

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Characteristics, risk factors and treatment reality in livedoid vasculopathy – a multicentre analysis

Weishaupt

Strölin

Kahle³

et al. 2019

Acad Dermatol Venereol

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105

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Background Livedoid vasculopathy (LV) is a rare cutaneous thrombotic disease. It is characterized by occlusion of dermal vessels resulting in livedo racemosa, ulceration and atrophie blanche. Clear guidelines for diagnosis and treatment are missing. Objective The purpose of this study was to better characterize epidemiology, clinical appearance and treatment reality of LV in a well‐defined patient cohort. Methods The cohort was allocated within a prospective, multicentre, phase IIa trial that investigated the effect of rivaroxaban in LV. Results Analysis of 27 patients revealed that LV patients had an increased Body Mass Index (BMI; 11/27), hypertension (19/27) and increased levels of lipoprotein (a) (5/12) and homocysteine (10/12) in the blood. The female‐to‐male ratio was 2.1 : 1, and the median age was 53.0 years [interquartile range (IQR) 40.5–68]. Investigation of the clinical appearance found that 82% of patients had livedo racemosa, and the ankle region was most likely to be affected by ulceration (56–70%). The analysis of patient treatment history showed that heparin was most effective (12/17), while anti‐inflammatory regimens were, although often used (17/24), not effective (0/17). Conclusion We add clinical clues for a data supported diagnosis of LV, and we provide evidence that anticoagulants should be administered in monotherapy first line (EudraCT number 2012‐000108‐13‐DE).

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Section: Discussionsupporting

confidence: 92%

“…The median age of the study population was 53 years (IQR: 40.5–68). Both facts are similar to data published by Renner et al . while others had described a female‐to‐male ratio of 3 : 1 and a clearly younger population with a mean age of 32 or 37 years .…”

Section: Discussionsupporting

confidence: 91%

Characteristics, risk factors and treatment reality in livedoid vasculopathy – a multicentre analysis

Weishaupt

Strölin

Kahle³

et al. 2019

Acad Dermatol Venereol

Self Cite

105

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“…Häufig finden sich hier serologisch erhöhte Kalzium‐ oder Phosphatwerte. Neben den renalen Erkrankungen gelten ein Vorliegen eines Hyperparathyreoidismus, Albuminmangel, Adipositas und Einnahme von Vitamin‐D3‐Analoga als Risikofaktoren . Differenzialdiagnostisch muss insbesondere das Ulcus cruris hypertonicum Martorell abgegrenzt werden.…”

Section: Calciphylaxieunclassified

Systemische Therapien des Ulcus cruris

Dissemond

Erfurt‐Berge

Goerge

et al. 2018

J Deutsche Derma Gesell

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ZusammenfassungFür eine erfolgreiche Behandlung von Patienten mit Ulcus cruris ist es essentiell, die zugrundeliegende Ätiologie zu klären und, wenn möglich, eine kausale Therapie einzuleiten. Begleitend ist die an den Phasen der Wundheilung orientierte Wundbehandlung, meist in Verbindung mit einer Kompressionstherapie, die Therapie der Wahl. Der Stellenwert der systemischen Therapien wird in Abhängigkeit der zugrundeliegenden Genese sehr unterschiedlich und oft kontrovers beurteilt. In diesem Übersichtsartikel werden die aktuellen Aspekte der systemischen medikamentösen Therapien für Patienten mit Ulcus cruris bei den häufigen Genesen chronische venö-se Insuffizienz, periphere arterielle Verschlusskrankheit, Livedovaskulopathie, Vaskulitis, Necrobiosis lipoidica, Calciphylaxie und Pyoderma gangraenosum differenziert vorgestellt und kritisch diskutiert.Zusammenfassend kann festgestellt werden, dass es sich bei vielen der hier vorgestellten Therapien um einen Off-Label-Use handelt. Bei den häufiger vorkommenden Wundarten wie beispielsweise Ulcus cruris venosum, mixtum und arteriosum sind Medikamente für die Therapie zwar hoffnungsvolle Optionen, bisher aber in der Regel kein Behandlungsstandard. Im Gegensatz hierzu haben systemische Therapien bei den anderen hier vorgestellten Krankheitsbildern eine meist zentral wichtige Bedeutung. Es sind in erster Linie immunmodulierende und rheologisch wirksame Medikamente, die eingesetzt werden, um die Wundheilung zu beschleunigen. SummarySuccessful management of patients with leg ulcers requires identification of the underlying etiology, with subsequent initiation of causal treatment, if feasible. Supplementary measures of first choice include stage-adjusted wound treatment, usually combined with compression therapy. The significance of systemic drugs has been the subject of controversial debate, depending on the underlying cause of the condition. The present review article is therefore meant to highlight current aspects of systemic drug therapies for the treatment of leg ulcers associated with chronic venous insufficiency, peripheral arterial disease, livedoid vasculopathy, vasculitis, necrobiosis lipoidica, calciphylaxis and pyoderma gangrenosum.In summary, the majority of therapeutic options presented herein are used off-label. While systemic drugs are promising options for the more common types of wounds such as venous, mixed or arterial leg ulcers, they do not represent the

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“…Next to end-stage renal disease and its co-morbidities like anemia and cardiovascular diseases, associations of CP with diabetes mellitus have been described. 16 Moreover, smaller numbers of reports have been published about possible associations between CP and autoimmune disorders, infectious or alcoholic hepatitis, antiphospholipid syndrome or duration of dialysis. 15 Chronic inflammatory states are discussed as possible risk factors for vascular calcification as TNF-a induces an osteogenic phenotype of human smooth-muscle cells in vitro.…”

Section: Risk Factors and Co-morbiditiesmentioning

confidence: 99%

<p>Management of patients with calciphylaxis: current perspectives</p>

Erfurt‐Berge¹,

Renner²

2019

CWCMR

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Calciphylaxis (CP) is a rare disorder presenting with painful ischemic skin ulcerations. Its association with end-stage renal disease, but also non-uremic cases have been described. Several risk factors, like metabolic syndrome, calcium-phosphate imbalance, anticoagulative medication and female gender have been discussed. Multidisciplinary therapeutic approaches are necessary since evidence-based guidelines are missing due to lack of pathophysiological understanding of the disease and the absence of studies with large patient cohorts. However, strategies for local wound therapy, reduction of risk factors and systemic therapies are being developed. Changes in duration, frequency and method of hemodialysis and specific medication to lower the calcium upload can be effective in uremic CP cases. Systemic treatments with cinacalcet, sodium thiosulfate or bisphosphonates have been applied successfully in CP patients, but large placebo-controlled randomized trials are still needed to gather better insight into this fatal disease.

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Analysis of the German DRG data for livedoid vasculopathy and calciphylaxis

Abstract: Our data analysis shows relevant comorbidity and cofactors of these rare diseases like livedoid vasculopathy and calciphylaxis in Germany by a large number of cases.

Cited by 17 publications

References 34 publications

Characteristics, risk factors and treatment reality in livedoid vasculopathy – a multicentre analysis

Characteristics, risk factors and treatment reality in livedoid vasculopathy – a multicentre analysis

Systemische Therapien des Ulcus cruris

<p>Management of patients with calciphylaxis: current perspectives</p>

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