Treating A Patient With Sepsis And Full Thickness Necrosis
A 43-year-old African American female went to the ICU at our institution with Streptococcus pneumoniae sepsis, which was complicated by disseminated intravascular coagulation.
The patient developed extensive ecchymotic lesions on her lower extremities. These lesions progressed to tender hyperpigmented macules with surrounding erythema, subsequently turned into large bullous lesions and eventually led to full thickness necrosis extending from the tibial tuberosity to the digits bilaterally.
A skin biopsy confirmed the diagnosis. In addition to receiving levofloxacin (Levaquin, Janssen Pharmaceuticals) and vancomycin for Streptococcus pneumoniae sepsis, the patient began IV heparin and warfarin (Coumadin, Bristol-Myers Squibb) for anticoagulation and to maximize tissue perfusion.
Wound care consisted of surgical and enzymatic debridement of necrotic tissue. The final surgical intervention included debridement of all remaining necrotic tissue, digital amputations and application of synthetic wound grafts.
Key Questions To Consider
1. What are the main characteristics of this condition?
2. What is the most likely diagnosis?
3. What is your differential diagnosis?
4. How can one make a definitive diagnosis?
5. What is the treatment?
Answering The Key Diagnostic Questions
1. The main characteristics of purpura fulminans are cutaneous hemorrhage and necrosis, most commonly associated with severe infection.
2. The most likely diagnosis is acute infectious purpura fulminans.
3. Differential diagnoses include thrombotic thrombocytopenic purpura, Henoch-Schönlein purpura and post-infectious thrombocytopenic purpura.
4. Definitive diagnosis of purpura fulminans occurs via skin biopsy.
5. Treatment of purpura fulminans is complex and one must tailor it to individual patients. Supportive treatment and the replacement of deficient blood components with fresh frozen plasma and clotting factors are the mainstays of treating this disorder.
What You Should Know About Purpura Fulminans
Purpura fulminans is a rare disorder of rapidly progressive cutaneous hemorrhage and necrosis of the skin. It is characterized by a petechial rash, which progresses to large, rapidly spreading skin hemorrhages and skin necrosis. It frequently occurs along with disseminated intravascular coagulopathy, fever and shock. The most common cause of purpura fulminans is severe infection but this disorder has also occurred in association with benign infections and deficiency of natural anticoagulants protein S and protein C.1 The pathogenesis of purpura fulminans results from the impairment of natural anticoagulant and fibrinolytic pathways, and activation of procoagulant pathways. Purpura fulminans most commonly occurs in children with fewer reported cases in adults.2-5
There are three types of purpura fulminans classified by their triggering mechanism: neonatal, idiopathic and acute infectious.2 Neonatal purpura fulminans is associated with a hereditary deficiency in anticoagulant factors protein C, protein S and antithrombin III. Idiopathic purpura fulminans most commonly happens after a benign illness. This form is the rarest type of this disorder and arises most commonly in pediatric patients. In idiopathic purpura fulminans, thrombi formation remains localized to the skin without organ involvement.6
Acute infectious purpura fulminans represents the most common form of this disorder. It most commonly occurs with meningococcal sepsis but authors have also reported this condition in association with other bacterial and viral infections.7 Acute infectious purpura fulminans is characterized by large purpuric skin lesions, fever, hypotension and disseminated intravascular coagulation.