Septic vasculitis I77.6

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 24.08.2022

Synonym(s)

Septic vasculitis, septic vasculitis; Vasculitis and sepsis; Vasculitis in sepsis

Definition
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Rare skin symptoms in sepsis caused mainly by meningococcus, gonococcus and Pseudomonas aeroginosa, either by haematogenic pathogen seeding or infectious-allergic or toxic (bacterial toxins) etiology. Mostly occurs in immunocompromised patients.

Pathogen
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Gonococci, meningococci, streptococci in the setting of endocarditis lenta, pseudomonads.

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Dermatology

Purpura fulminans

Rare acquired syndrome following an initial infectious episode with consumption coagulopathy, haemorrhages ...

Etiopathogenesis
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Bacterial decomposition products (endotoxins, superantigens) or released mediators cause fatal damage to the vessel wall with unphysiological activation of the coagulation system (disseminated intravascular coagulation; consumption coagulopathy).

Manifestation
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Occurs in young adults of both sexes.

Localization
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Predominantly acral lesions.

Clinical features
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Usually accompanied by life-threatening, severe general symptoms (high fever, somnolence, drop in blood pressure, tachycardia), shooting up within a few hours, acrally localized, widely spread, 1-2 mm large hemorrhagic spots or papules, more rarely pustules with erythematous margins. Often central necrosis, also splinter hemorrhages.

See below for details. Purpura fulminans, Waterhouse-Friderichsen syndrome, consumption coagulopathy,

S.a. under sepsis, skin lesions.

Laboratory
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Signs of consumption coagulopathy, fibrinolysis, thrombosis and bleeding are to be searched for.

Furthermore, determination of fibrin monomers, fibrinopeptide A (indicating intravascular coagulation), D-dimers (reactive hyperfibrinolysis), platelets, fibrinogen and antithrombin (indicating severity of consumption coagulopathy).

Histology
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Conspicuous are hyaline thrombi in the lumen of postcapillary venules. Perivascular oedema; erythrocyte extravasations. Moderately dense, superficial, perivascularly stored, inflammatory infiltrate of neutrophil granulocytes, lymphocytes, more rarely plasma cells. The epidermis may show incipient necrosis. Bacterial clusters in the vascular lumens are rarely detectable. The exudation can be so strong that subepidermal blistering can occur.

Differential diagnosis
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Leucocytoclastic vasculitis; vasculitis with essential cryoglobulinemia; disseminated intravascular coagulopathy (see coagulopathies below).

Therapy
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Immediate intensive care: treatment of septic shock and consumer coagulopathy.

Progression/forecast
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Very serious. Fatal course is possible within hours (multiorgan failure). Chronic recurrent courses, in which the described severe general symptoms are missing, are described.

Note(s)
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In case of foudroyantly developing large-area purpura with rapidly deteriorating AZ, sepsis must be considered.

Literature
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Sundkötter C et al (2004) Leucocytoclastic vasculitis. Dermatologist 55: 759-783
Tomasini C (2015) Septic vasculitis and vasculopathy in some infectious emergencies: theperspective
of the histopathologist. G Ital Dermatol Venereol 150:73-85.