Purpura fulminans in a patient with mixed connective tissue disease.
A 43-year-old lady was admitted to the intensive care unit with sepsis. She had a history of mixed connective tissue disease, Raynaud's syndrome and hypothyroidism. 2 days later, she developed a purpuric rash on her face and extremities with a livedoid background. Few days later, her distal fingers and toes became gangrenous which then had to be amputated. Laboratory investigations showed that she was coagulopathic and had multiple organ dysfunctions. Antiphospholipid antibodies were negative; however, protein C and antithrombin III levels were low. A skin biopsy showed fibrinoid necrosis in the vessel wall with microthrombi and red-cell extravasation. A diagnosis of purpura fulminans was made.
Dermatology Department,  Adelaide and Meath Hospital, Dublin,  Ireland.
Aizuri A
AA
Murad
Michael
M
Jeffers
Anne-Marie
AM
Tobin
Maureen
M
Connolly
D000328:Adult; D000925:Anticoagulants / Q000627:therapeutic use; D003937:Diagnosis, Differential; D005145:Face; D005260:Female; D005385:Fingers; D006801:Humans; D008947:Mixed Connective Tissue Disease / Q000150:complications*; D011486:Protein C / Q000627:therapeutic use; D055665:Purpura Fulminans / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy / Q000473:pathology; D018805:Sepsis / Q000150:complications; D012867:Skin / Q000473:pathology; D014034:Toes
D000925:Anticoagulants; D011486:Protein C