UPMC Dermatopathology "Case of the Month" Presentations
UPP - Department of Dermatology, Dermatopathology Unit
Pooja Khera, MD; Laura Ferris, MD; Arash Radfar, MD, PhD
FEBRUARY 2007 CASE OF THE MONTH
CLINICAL FINDINGS
MB is a 32-year old white female with a history of Hodgkin’s lymphoma status post chemotherapy, radiation, and splenectomy as well as transfusion-related HIV. Her last CD4 count was 730. She had fever, chills, myalgias, nausea, and vomiting one day prior to admission. The following morning, she experienced acute onset chest pain and dyspnea for which she was taken to the Emergency Department. She also noted a rash associated with burning pain on her lower extremities. The patient denied sick contacts and recent travel. As far as she knew, she had not received vaccinations for encapsulated organisms after her splenectomy 15 years ago. She had never been on HAART therapy and was on no medications at the time of admission.
On physical examination, the patient’s vitals were: Temperature 38.5, HR 111, RR 14, BP 95/60. The patient was in significant distress and had petichiae, ecchymoses, and edema of her face and neck (Figure 1). She had gingival bleeding. She had a reticulated pattern of non-palpable purpura covering her legs bilaterally with few overlying flaccid bullae. There was no evidence of gangrene or necrosis (Figures 2,3).
Her laboratory values were as follows: Hb 13.3, Hct 38, WBC 6.8 with 46% neutrophils, 15% lymphocytes, and 36% bands, plts 28. BUN 22, Cr 2.2, HCO3 8, lactate 15. PT 19, PTT 110, INR 1.9, DIC panel showed elevated fibrin degradation products and decreased fibrinogen. A CT scan revealed bilateral adrenal hemorrhages.
Blood cultures for bacteria and fungi remained negative throughout the hospital course. Protein C, protein S, Antithrombin III, and Factor V Leiden were all within normal limits. A punch biopsy of the skin was performed.
A 4 mm punch biopsy revealed extensive epidermal necrosis with occlusion of the underlying dermal venules and capillaries by microthrombi (Figures 4,5,6). In addition, there was significant hemorrhage into the dermis with a sparse superficial perivascular inflammatory infiltrate composed of lymphocytes and rare neutrophils (Figure 7). A PAS stain, Gram stain, and varicella-zoster immunostain failed to reveal microorganisms. Overall, the histological findings of a thrombo-embolic microangiopathy with overlying epidermal necrosis were consistent with the clinically suspected purpura fulminans.
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