Purpura fulminans D65.x

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 06.04.2021

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Synonym(s)

Purpura Enoch

History
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Henoch, 1874; Glanzmann, 1918

Definition
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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.

Classification
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  • 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)

Etiopathogenesis
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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.

Manifestation
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Mostly infants, also adults.

Clinical features
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Severe general symptoms. Extensive and petechial haemorrhages and blisters in symmetrical arrangement on the extremities, face and trunk, rapidly changing into necroses.

Laboratory
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Determination of protein C (<25%).

Therapy
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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/forecast
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Awkward. High lethality due to bleeding into internal organs.

Literature
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  1. Auletta MJ et al (1988) Purpura fulminans. Arch Dermatol 124: 1387-1391
  2. 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
  3. Elliott MA et al (2001) Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Mayo Clin Proc 76: 1154-1162
  4. Feldmann R et al (1991) The significance of protein C deficiency in dermatology. Dermatologist 42: 607-612
  5. Glanzmann WE (1918) Hereditary haemorrhagic thrombasthenia. A contribution to platelet pathology. Yearbook for Pediatrics 88: 1-42 and 113-141
  6. Enoch E (1874) On a peculiar form of purpura. Berliner klin weekday 11: 641
  7. Lubach D et al (1984) Two-dimensional purpura with necroses in severe cases of consumption coagulopathy. Dermatologist 35: 152-158
  8. 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
  9. Scheinfeld N (2003) Fatal fulminant hypereosinophilic syndrome with a petechial rash in a 16-year-old girl. J Pediatric Hematol Oncol 25: 827-830

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 06.04.2021