Bullae and purpura revealing protein C consumption

We present two patients’ clinical images showing severe foot lesions characterized by bullous appearance over a diffusely purpuric skin, with serious fingertip involvement. Figure 1a and b presents a 4-year-old child suffering from Streptococcus pyogenes septic shock while Fig. 1c and d shows an 18-month-old child with group C Neisseria meningitidis septic shock. Both cases were complicated by severe DIC (with admission protein C level equal to 8 and 2%, respectively) followed by massive rhabdomyolysis and myoglobinuria; low-dose fenoldopam maintained renal function in the first case, whereas hemodiafiltration was necessary in the second patient. Both children survived after receiving high-level hemodynamic and ventilatory support. Cutaneous anomalies characterized by the presence of purpura and bullae may be due to several causes with different pathophysiology, clinical presentation, and location distinguishing each clinical form. Differential diagnoses include DIC, sepsis, Henoch-Scho¨nlein purpura, necrotizing fasciitis, systemic lupus erythematosus, hemorrhagic erysipelas, bullous amyloidosis, ecthyma gangrenosum, thrombocytopenia, gloves and socks syndrome, and herpetiform dermatitis [1–4]. In our cases, cutaneous lesions resulted from massive ischemic processes involving lower limb terminal districts. Distal perfusion was improved by treating the thrombotic process through protein C concentrate [5] and antithrombin massive replacement; intravenous pentoxifylline and low-dose prostacyclin were given due to their antiinflammatory and rheologic properties. Eventually, only a few distal phalanges were partially lost in both patients. Massive rhabdomyolysis and myoglobinuria were treated conservatively for the meningococcal sepsis patient, whereas a 6-day course of continuous veno-venous hemodiafiltration was needed for the Streptococcus pyogenes- affected child.