Purpura Fulminans

THOMASE. SPGER,M.D.’ JEROLDM. RAU, M.D. Kansas City, Kansas

Purpura fulminans is a rare disease characterized by cutaneous ecchymosis, hypotension and fever associated with disseminated intravascular coagulation. It usually follows a preparatory infectious illness. A case in a 55 year old woman challenges the concept that this disease is limited to childhood. A review of 100 case reports is presented in which the oflen quoted high mortality of 90,per cent is found to be no longer tenable. Purpura fulminans is generally reported to be a rare disease of childhood with a mortality rate approaching 90 per cent. The three primary clinical features of this syndrome, which follows a nonspecific preparatory, usually infectious, illness, are: (1) large purpuric ecchymotic areas of the skin, (2) fever and (3) hypotension. Characteristically, the hematologic picture is one of consumption coagulopathy; the pathologic picture is one of venule and capillary thrombosis, involving primarily the skin, but occasionally other organs. The pathogenesis of the disease, although never fully elucidated, closely parallels the generalized Shwartzman reaction of laboratory animals, It is our purpose here to report an unusual case of purpura fulminans in a 55 year old white woman and to review the literature to more clearly define the confines of the syndrome.

CASE REPORT

From the Departmentof Medicine, Section of Dermatology,Universityof KansasMedicalC&nter, 29th and Rainbow Boulevard,KansasCity, Kansas66103. Requestsfor reprints should be addressed to Dr. Jerold M. Rau. Manuscript accepted January 9, 1976. “Present address: 3480 East 3020 South, Salt Lake City, Utah 84 109.

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This patient was referred to the University of Kansas Medical Center for admission because of a sudden onset of shock and associated purpura. Ten days prior to admission, she had the onset of a febrile upper respiratory tract illness for which she was hospitalized at another facility. Symptomatically improved on conservative therapy, including oral erythromycin estolate, the patient was discharged after three days. Five days later, she began to experience persistent abdominal pain, followed in two days by diarrhea, for which she was given an opiate for symptomatic relief. Ten days after discharge, a rapidly spreading petechial rash appeared, first on the dorsum of the feet and then spreading to become ecchymotic involving the legs to the mid-thigh, the arms, abdomen and face. On the morning of admission, she presented to her personal physician in shock. Her past medical history revealed multiple drug hypersensitivities including opiates, A variety of cutaneous eruptions had previously occurred with the use of multiple antibiotics, but the patient denied any previous reaction to erythromycin. Physical examination revealed a white woman who was confused but oriented. He: blood pressure was 60/40 mm Hg , pulse rate 140 beats/min, respirations 24/min, and temperature 38’C rectally. Examination of her skin disclosed violaceous ecchymoses, which were nonraised and nontender over the areas previously noted. Examinations of the head and neck disclosed no abnormalities. There were no conjunctival, retinal or oral hemorrhages. Examination of the chest and heart showed no abnormalities other than hypotension with tachycardia as already noted. Bowel sounds were hypoactive, but present. Rectal ex-

October 1976 The American Journal of Medlclne

Volume 61

PURPURA FULMINANS-SPICER,

amination produced a small amount of bloody mucous. There were no focal neurologic signs. Laboratory data disclosed a hemoglobin level of 14.2 g/dl, falling to 6.8 g/dl despite a 12 unit transfusion. The white cell count was 23,900/mm3 with 50 per cent segmented forms, 29 per cent band forms, 5 per cent lymphocytes and 4 per cent metamyelocytes. Platelet count was 5 1,000/mm3. Examination of the peripheral blood smear demonstrated an occasional schistocyte. Fibrinogen level on admission was 139 mg/dl (normal 200 to 400 mg/dl). Prothrombin time was 16.3 set with a control of 11.1 set, and activated partial thromboplastin time was 66.3 set (normal 30 to 40 set) which corrected to normal with a I:1 mix with normal plasma. Thrombo Wellco was positive for fibrin degradation products greater than 40 pg/ml. Urinalysis revealed 4-t protein, 25 to 50 white blood cells per high power field (hpf), 10 to 20 red blood cells/hpf, l-t bilirubin and 1-F hemoglobin. Examination of sternal marrow aspirate disclosed myeloid hyperplasia with an increased number of megakaryocytes. The patient’s intial course was complicated by shock responsive to volume expansion with electrolyte and protein solutions. Two units of whole blood and 10 units of packed red cells were required to maintain the hemoglobin level. Abdominal pain was evaluated by a surgical consultant who, on proctosigmoidoscopy, observed ecchymotic mucosal changes and performed a rectal biopsy with some subsequent bleeding in the lower gastrointestinal tract. A hematologic picture of disseminated intravascular coagulation was apparent but bleeding in the gastrointestinal tract precluded early heaprinization. Bleeding was controlled by the seventh hospital day, and heparinization at that point produced a remarkable rebound in platelet count, and fibrinogen levels approached normal (see Figure 1). The administration of heparin was discontinued on the 13th hospital day and those

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Purpura fulminans.

Purpura Fulminans THOMASE. SPGER,M.D.’ JEROLDM. RAU, M.D. Kansas City, Kansas Purpura fulminans is a rare disease characterized by cutaneous ecchymo...
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