Venous Leg Ulcers in a Patient with Klinefelter's Syndrome and Increased Activity of Plasminogen Activator Inhibitor-1

Dissemond, Joachim; Schultewolter, T.; Brauns, Tilmann C.; Goos, M.; Wagner, Stephan N.

doi:10.1080/00015550310007607

Cited by 5 publications

(4 citation statements)

References 15 publications

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“…2 venous leg ulcers and elevated levels of PAI-1 activity in Klinefelter's syndrome has been discussed. 8,9 As Klinefelter's syndrome is known to be associated with a relative androgen deficiency a bilateral orchidectomy in rats has been shown to induce an increase in activity of coagulation factors such as PAI-1. The administration of testosterone results in venous ulcer healing and stimulates fibrinolysis.…”

Section: Discussionmentioning

confidence: 99%

“…Local impairment of fibrinolysis leading to the development of microthrombi may be other important factors in these thromboembolic processes 2 . Moreover, a causal relationship between venous leg ulcers and elevated levels of PAI‐1 activity in Klinefelter's syndrome has been discussed 8,9 . As Klinefelter's syndrome is known to be associated with a relative androgen deficiency a bilateral orchidectomy in rats has been shown to induce an increase in activity of coagulation factors such as PAI‐1.…”

Section: Discussionmentioning

confidence: 99%

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Increased activity of factor VIII coagulant associated with venous ulcer in a patient with Klinefelter's syndrome

Dissemond

Knab

Lehnen

et al. 2005

Acad Dermatol Venereol

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Klinefelter's syndrome is the most frequent major abnormality of sexual differentiation in men with two or more X chromosomes. Recurrent venous ulcers as a result of a post-thrombotic syndrome are a well known symptom in patients with Klinefelter's syndrome. Until now the underlying pathomechanisms are not completely understood. Platelet hyperaggregability, factor V Leiden mutation and abnormalities in fibrinolysis were implicated as possible contributing factors. Here we describe the detection of an increased activity of factor VIII coagulant (factor VIII:C). This is the first case report on increased factor VIII:C activity associated with venous ulcers in a patient with Klinefelter's syndrome. Elevated factor VIII plasma levels are gradually accepted to be associated with an increased risk for venous thromboembolism. Therefore, we discuss that the examination of factor VIII:C may help in clarifying individual thromboembolic risks, especially in patients with Klinefelter's syndrome.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Increased activity of factor VIII coagulant associated with venous ulcer in a patient with Klinefelter's syndrome

Dissemond

Knab

Lehnen

et al. 2005

Acad Dermatol Venereol

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“…Von den zahlreichen potenziell mit dem Auftreten eines Ulcus cruris assoziierten genetischen Erkrankungen (16) (13). Somit kommt es gehäuft zu einem Ulcus cruris postthromboticum.…”

Section: Genetische Defekteunclassified

Differenzialdiagnosen des Ulcus cruris venosum

Dissemond¹

2011

Phlebologie

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ZusammenfassungEs wird geschätzt, dass in Deutschland etwa 1% der erwachsenen Bevölkerung an einem Ulcus cruris unterschiedlichster Genese leidet. Auch wenn die chronische venöse Insuffizienz und die periphere arterielle Verschlusskrankheit bei etwa 80% aller Patienten mit einem Ulcus cruris pathophysiologisch relevant sind, so ist doch die Kenntnis der relevanten Differenzialdiagnosen insbesondere bei therapierefraktären Verläufen von entscheidender Be-deutung. Es existieren neben Vasculitiden auch neuropathische, metabolische, hämatologische und exogene Faktoren sowie Neoplasien, Infektionen, Medikamente, genetische Defekte und primäre Dermatosen, die ein Ulcus cruris verursachen können. Bei vielen Pa-tienten können es auch multiple Faktoren sein, die zu einem Ulcus cruris führen bzw. dessen Abheilung trotz scheinbar adäquater Behandlung verhindern. Als relevante Ko-Faktoren können hier beispielsweise Adipositas, Diabetes mellitus, Ekzeme, (Lymph-)Ödeme oder arterielle Hypertonie eine bedeutende Rolle spielen.Für eine dauerhaft erfolgreiche Behandlung der Patienten mit einem Ulcus cruris ist es so-mit von entscheidender Bedeutung alle relevanten Faktoren der Pathogenese zu diagnostizieren, um eine kausal ansetzende, dauerhaft erfolgreiche Therapie zu gewährleis-ten.

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“…Leg ulcers related to PTS are reported in 6-13 % of affected patients. Proposed causal factors include various thrombogenic factors such as vascular deformities, increased thrombocyte aggregation, increased factor VIII activity, or elevation of plasminogen activator-inhibitor (PAI)-1 in patient serum [23,24].…”

Section: Genetic Defectsmentioning

confidence: 99%

Differential diagnosis of leg ulcers

Dissemond

Körber

Grabbe

2006

J Deutsche Derma Gesell

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In Germany about 0.7% of the adult population have a chronic leg ulcer. Although chronic venous insufficiency accounts for at least 80% of all chronic leg ulcers, knowledge of the relevant differential diagnostic considerations is of crucial importance, in particular for patients who are refractory to therapy. In addition to vascular disease, other causes include neuropathic, metabolic, hematologic and exogenous factors as well as neoplasias, infections, drugs, genetic defects and some primary skin disorders. For the long-term successful treatment of patients with chronic leg ulcers, it is necessary to identify all relevant factors, in order to enable a pathogenesis-oriented, interdisciplinary therapeutic approach.

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Venous Leg Ulcers in a Patient with Klinefelter's Syndrome and Increased Activity of Plasminogen Activator Inhibitor-1

Cited by 5 publications

References 15 publications

Increased activity of factor VIII coagulant associated with venous ulcer in a patient with Klinefelter's syndrome

Increased activity of factor VIII coagulant associated with venous ulcer in a patient with Klinefelter's syndrome

Differenzialdiagnosen des Ulcus cruris venosum

Differential diagnosis of leg ulcers

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