Author(s): Dr Danica Xie, Dermatology Senior Resident Medical Officer; Dr Russell Thompson, Dermatology Registrar; and Dr Alicia Thornton, Consultant Dermatologist, John Hunter Hospital, NSW, Australia
Reviewing dermatologist: Dr Ian Coulson (2024).
Edited by the DermNet content department
A 53-year-old female was admitted to the intensive care unit with septic shock. Past medical history included acute lymphoblastic leukaemia in remission, stem cell transplant with rejection, pulmonary embolism, and cerebrovascular accident.
The patient developed widespread petechiae and non-blanching purpura on her face, trunk, and limbs. Overnight she developed small blisters on her lower legs and oral erosions. Over subsequent days dry gangrene developed affecting her nose, fingers, and feet.
Investigations confirmed thrombocytopenia with platelets 17x10^9/L (reference range 150–400x10^9/L) and low fibrinogen 1.0g/L (reference range 2.0–4.6g/L).
Empirical antibiotics were commenced. Subsequently blood cultures and urinary antigens detected Streptococcus pneumoniae.
Purpura fulminans: a rare, rapidly progressive and often fatal form of acute disseminated intravascular coagulation (DIC). It is marked by intravascular coagulation, cardiovascular shock, and haemorrhagic infarction of the skin.
Purpura fulminans can be idiopathic, or associated with serious infection, mostly commonly Neisseria meningitidis, Streptococci, and methicillin-resistant Staphylococcus aureus (MRSA).
Definitive diagnosis is with:
Investigations for causes of thrombophilia should be ordered, such as protein C and protein S (deficiency) and antithrombin III.
Prompt recognition of this entity is required to institute timely life-saving measures. Urgent admission to an intensive care unit (ICU) is paramount.
Multidisciplinary input is required for:
