Purpura fulminans mit ausgedehnten Hautnekrosen

Abstract

Purpura fulminans must be treated as an emergency in internal medicine and dermatology. Its characteristic features are the sudden development of progressively enlarging haemorrhagic skin necrosis, severe disseminated intravascular coagulation with consumption of anticoagulant factors, and signs of shock. Purpura fulminans can be classified into a neonatal form with inherited protein C deficiency and an acquired type for which multiple causes are known. Clinically it is characterized by massive ecchymosis with haemorrhagic blebs and acral necrosis. Histologically the lesions show widespread extravasation of erythrocytes and thrombosis of small vessels. Thrombocytopenia, decrease of coagulation factors, the presence of fibrinogen split products and fragmented erythrocytes in the blood smear help to confirm the diagnosis. The therapy includes fresh-frozen plasma, heparin, antibiotics and surgical debridement of necrotic areas. It is important to recognize the disease promptly because the mortality rate is about 30-40% and only quick intervention helps to save the life of the patient.

Purpura fulminans des Erwachsenen – ein hämatologischer Notfall

“…Ursächlich für den Protein-C-Mangel sind Infektionen, die zu einer Endotoxin-Freisetzung führen. Dadurch wird das Gerinnungssystem aktiviert und eine disseminierte intravasale Gerinnung ausgelöst [11]. Nach bisherigem Kenntnisstand sind hämorrhagische Diathesen typisch, die sich insbesondere an den unteren Extremitäten, dem männlichen Genitale sowie dem Rücken manifestieren.…”

“…Nach bisherigem Kenntnisstand sind hämorrhagische Diathesen typisch, die sich insbesondere an den unteren Extremitäten, dem männlichen Genitale sowie dem Rücken manifestieren. Gesicht und Körperstamm sind eher selten betroffen [11]. Die Hauteinblutungen gehen nach 24-48 h in Nekrosen (q Abb.…”

Skin diseases in intensive care medicine

“…An important differential diagnosis of allergic vasculitis with palpable purpura is purpura due to thrombocytopenia or thrombocytopathy, which in contrast is usually not palpable. The clinical presentation of purpura fulminans which begins with small maculae that rapidly expand to extensive, bizarrely configured skin hemorrhages, often with hemorrhagic blisters as an expression of necrosis, must be differentiated [48] (Figure 5). Features resemble disseminated intravascular thrombosis, characterized histologically by thromboses of dermal blood vessels [48].…”

“…The clinical presentation of purpura fulminans which begins with small maculae that rapidly expand to extensive, bizarrely configured skin hemorrhages, often with hemorrhagic blisters as an expression of necrosis, must be differentiated [48] (Figure 5). Features resemble disseminated intravascular thrombosis, characterized histologically by thromboses of dermal blood vessels [48]. Causes are numerous, with viral and especially bacterial infections (Table 3) being of particular significance in the intensive care setting.…”