Presence of rash in a catastrophic condition.

Dear Editor,

We present a case of purpura fulminans in a patient with septic shock. A 43-year-old male presented with severe abdominal pain, vomiting, and diarrhea, all of which occurred shortly after eating at a restaurant. His history was significant for diabetes mellitus and splenectomy after a motor vehicle accident. On physical examination, he was alert but was tachypneic, tachycardic, and hypotensive, requiring norepinephrine infusion. He had a diffuse bluish-purple discoloration of the face, in addition to a mottled appearance over his trunk and extremities with erythematous papules [Figures 1 and 2]. The rash consisted of non-blanching purpuric lesions with areas of necroses. Laboratory workup revealed leukocytosis, high anionic gap acidosis, renal failure, and consumptive coagulopathy. Subsequently, the patient developed acute mental mental status changes, respiratory distress requiring intubation and severe septic shock ultimately leading to his demise.

Figure 1

Bluish-purple purpura over face with necrotic areas (denoted by arrows)

Figure 2

Mottled reticular rash with red papules (arrowhead) over lower extremity

Our patient exhibited purpuric lesions, suggestive of acute purpura fulminans along with cardinal signs of sepsis; which is the most common cause of these types of lesion. The origin of septic shock was possibly endotoxins in his food as blood culture did not grow any organism. The presence of purpura fulminans heralds a poor outcome in these patients.

Purpura fulminans has three variants[1] — neonatal purpura fulminans seen in association with hereditary protein C or S or antithrombin III deficiency. Idiopathic purpura can occur in adults following a viral illness, acquired protein C or S deficiency and disseminated intravascular coagulation (DIC). However, the most common cause remains infectious purpura fulminans. It is seen with multitude of organisms, notably with staphylococcus, streptococcus, hemophilus, and pseudomonas.[2] Nevertheless, it occurs so commonly in the context of meningococcal infection that its presence is considered a cardinal feature of meningococcal septicemia. This condition is also more common in asplenic individuals because of sepsis due to encapsulated organisms.[3] In these cases, the exotoxins or endotoxins from the organisms activate cytotoxins such as interleukin 2, tumor necrosis factor-alpha which triggers the consumption of protein C, protein S, and antithrombin III, thus disturbing the balance of anticoagulant and procoagulant activities of the endothelial cells.

This condition typically manifests as erythematous purpuric macules with areas of blue-black hemorrhagic necroses in acral sites of the body, although macules can appear anywhere on the skin.[1] It is characterized by rapidly progressive skin necrosis and hemorrhage and extensive dermal intravascular thrombosis (identical to Shwartzman reaction, which is a necrotizing inflammatory lesion provoked by the gram-negative bacteria) in the setting of DIC and vascular collapse.[4]

Mortality with purpura fulminans is around 40%. The medical management mainly involves supportive care in Intensive Care Unit. Other therapeutic interventions such as activated protein C (APC) infusion, recombinant tissue plasminogen activator have been employed with variable results. The APC infusion is currently not licensed for use in purpura fulmians associated with sepsis. The use of heparin or corticosteroids remains controversial, because it does not improve survival. Plasmapheresis can be used in extreme cases. Recent advances in early surgical interventions in the form of compartment release, sympathectomy performed concurrently with the initial treatment of sepsis have improved outcomes.[5]