HistoryThis section has been translated automatically.
Henoch, 1874; Glanzmann, 1918
DefinitionThis section has been translated automatically.
Rare acquired syndrome following an initial infectious episode with consumption coagulopathy, haemorrhages in skin and internal organs and subsequent necroses. S.a. Waterhouse-Friderichsen syndrome.
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ClassificationThis section has been translated automatically.
- Congenital form (due to a congenital protein C deficiency): onset of symptoms 3 days after birth
- Acquired form: maximum form of the cutaneous form of sepsis and disseminated intravascular coagulopathy (DIC)
EtiopathogenesisThis section has been translated automatically.
Discussed are vascular wall changes caused by an infection allergy and a protein C deficiency acquired by the infection as an initiator for disseminated intravascular coagulation, which occur 1-4 weeks after an infection, e.g. with streptococci, meningocococci, Haemophilus influenzae, staphylococci, varicella or viral infections of the upper respiratory tract.
ManifestationThis section has been translated automatically.
Mostly infants, also adults.
Clinical featuresThis section has been translated automatically.
Severe general symptoms. Extensive and petechial haemorrhages and blisters in symmetrical arrangement on the extremities, face and trunk, rapidly changing into necroses.
LaboratoryThis section has been translated automatically.
Determination of protein C (<25%).
TherapyThis section has been translated automatically.
Intensive medical care with circulation stabilisation, fluid and electrolyte balancing. Stage-appropriate shock treatment. Immediate fibrinolytic therapy and heparinization in case of manifest disseminated intravascular coagulation. In addition, treatment of the primary, causative infection.
Progression/forecastThis section has been translated automatically.
Awkward. High lethality due to bleeding into internal organs.
LiteratureThis section has been translated automatically.
- Auletta MJ et al (1988) Purpura fulminans. Arch Dermatol 124: 1387-1391
- Chak WK et al (2003) Thrombotic thrombocytopenic purpura as a rare complication in childhood systemic lupus erythematosus: case report and literature review. Hong Kong Med J 9: 363-368
- Elliott MA et al (2001) Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Mayo Clin Proc 76: 1154-1162
- Feldmann R et al (1991) The significance of protein C deficiency in dermatology. Dermatologist 42: 607-612
- Glanzmann WE (1918) Hereditary haemorrhagic thrombasthenia. A contribution to platelet pathology. Yearbook for Pediatrics 88: 1-42 and 113-141
- Enoch E (1874) On a peculiar form of purpura. Berliner klin weekday 11: 641
- Lubach D et al (1984) Two-dimensional purpura with necroses in severe cases of consumption coagulopathy. Dermatologist 35: 152-158
- Manco-Johnson MJ et al (2004) Activated protein C concentrate reverses purpura fulminans in severe genetic protein C deficiency. J Pediatr Hematol Oncol 26: 25-27
- Scheinfeld N (2003) Fatal fulminant hypereosinophilic syndrome with a petechial rash in a 16-year-old girl. J Pediatric Hematol Oncol 25: 827-830
Incoming links (9)Neisseria meningitidis ; Protein c; Purpura enoch; Sepsis skin changes; Septic vasculitis; Small vessel vasculitis, cutaneous; Streptococcus; Varicella; Waterhouse friderichsen syndrome;
Outgoing links (2)Protein c; Waterhouse friderichsen syndrome;
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.