Livedovasculopathy
Livedovasculopathy (from livid 'bluish' and vasculopathy 'vascular disease') is a coagulation disorder of smaller blood vessels in the skin, which leads to very painful tissue collapse and skin necrosis . The result is irrevocable scars . Livedovasculopathy is a rare disease and is also known as livedo vasculitis , segmental hyalinizing vasculitis , livedo reticularis with summer ulcerations , atrophy blanche or PURPLE ( painful purpuric ulcers with reticular pattern of lower extremities ).
| Classification according to ICD-10 | |
|---|---|
| L95 | Livedo vasculitis |
| ICD-10 online (WHO version 2019) | |
General
Livedovasculopathy is a chronic, recurrent vascular disease affecting the microcirculation of the skin. Vascular occlusions occur in the small vessels of the upper skin layer, which lead to an undersupply of the overlying skin layer and thus trigger tissue destruction (skin infarction ). The result is very painful ulcerations (open wounds) on the skin that heal poorly. The disease is often recognized late but can be treated well.
root cause
In the small supply vessels of the skin, there is an increased willingness to clot ( thrombophilia ) for reasons that have not yet been fully clarified . Since areas of the topmost skin layer are only supplied by a few vessels, the vascular occlusion leads to an insufficient supply with tissue loss after a short time. This tissue destruction ( necrosis ) is painful. The disease occurs more frequently in the summer months. One possible explanation is that at higher temperatures, the fluid level in the blood drops, thereby increasing the viscosity, and thus increasing the willingness to clot. A distribution of 3: 1 with regard to the female gender is striking. Here, too, it is not yet clear why women are affected significantly more often than men.
Disease emergence
The occurrence of the disease is not yet fully understood. Studies on larger patient collectives were able to demonstrate altered coagulation factors in the blood of some patients, but these are neither conclusive for the diagnosis of livedovasculopathy nor disproving if the result is negative.
Clinical manifestations
Livedovasculopathy is characterized by a triad of symptoms . The so-called Livedo racemosa drawing is assessed as a predisposition for the disease. The acute ulceration ("open wound") is the manifestation of the disease in the acute episode. The atrophy blanche is the scarred residue that remains visible on the skin after the acute attack has subsided.
Investigation methods
The diagnosis of livedovasculopathy can only be made clinically. This means that only the synopsis of anamnesis (patient discussion) and clinical findings lead to a diagnosis. It turns out that particularly specialized outpatient departments at the university clinics are suitable for making the diagnosis. In Germany z. E.g. the Dermatology Clinic Münster in the field of live vasculopathy and offers not only comprehensive diagnostics but also innovative treatment methods.
pathology
The cause is excessive coagulation of the skin's microcirculation. The pain that occurs is the result of the insufficient supply of blood to the skin. If the insufficient supply lasts too long, tissue destruction occurs (skin infarction).
treatment
The treatment of livedovasculopathy aims to reduce the patient's willingness to clot. So drugs are used that are known from thrombosis prophylaxis. Low molecular weight heparins are mostly used in the form of "anti-thrombosis injections". In addition, a case series has already shown benefits from the use of rivaroxaban (Xarelto ©). The therapy should be individually coordinated with the patient in terms of dose and duration and is subject to strict consideration of the bleeding risk of the therapy. A study on the effectiveness of antithrombotic therapy in the treatment of lived vasculopathy is currently being carried out at several university hospitals in Germany.
prevention
Effective preventive measures to prevent livedovasculopathy are currently unknown. Nicotine consumption should generally be stopped in vasculopathies, as nicotine leads to further constriction of the blood vessels.
Prospect of healing
The lived vasculopathy can be controlled under therapy with anticoagulant drugs, in particular in many patients the pain subsides quickly. Since the disease can recur permanently, it is advisable to connect to a specialized center.
history
Feldaker and colleagues described the disease as livedo reticularis with summer ulceration as early as 1955. Since the late 1990s, the disease has been understood as vasculopathy (coagulation disease) and is thus different from inflammatory vasculitis.
See also
Web links
- www.livedovaskulopathie.de (further information on the disease and treatment options in Germany)
Individual evidence
- ↑ a b N. Kerk, T. Goerge: Livedoid vasculopathy - a thrombotic disease. In: VASA. 2013 Sep; 42 (5), pp. 317–322. Review.
- ↑ a b Tobias Goerge: Pathogenesis, diagnosis and treatment of cutaneous infarction. In: dermatologist. 2011 Aug; 62 (8), pp. 627-634; Review. German.
- ↑ a b c A. Alavi, J. Hafner, JP Dutz, D. Mayer, RG Sibbald, PR Criado, P. Senet, JP Callen, TJ Phillips, M. Romanelli, RS Kirsner: Livedoid vasculopathy: an in-depth analysis using a modified Delphi approach. In: J Am Acad Dermatol. 2013 Dec; 69 (6), pp. 1033-1042.
- ↑ a b N. Kerk, A. Drabik, TA Luger, SW Schneider, T. Goerge: Rivaroxaban prevents painful cutaneous infarctions in livedoid vasculopathy. In: Br J Dermatol . 2013 Apr; 168 (4), pp. 898-899.
- ↑ ( RILIVA )
- ↑ M. Feldaker et al: Livedo reticularis with summer ulcerations. In: AMA Arch Derm. 1955 Jul; 72 (1), pp. 31-42.
- ↑ JL Jorizzo: Livedoid vasculopathy: what is it? In: Arch Dermatol. 1998, 134, pp. 491-493.
