Lab results will show thrombocytopenia, hemolytic anemia, increases in prothrombin time and partial thromboplastin time, and an increase in fibrin degradation products. As the disease progresses, the fibrinogen level falls.
Treatment starts off similar to treating TTP, with an emergent consultation with hematology/oncology, ICU admission, and treatment of the underlying illness. Add supporting vitamin K and folate. "Then it gets tricky," she said. "You’re going to need to balance how much of their problem is from bleeding and not enough coagulation factors and platelets, and how much is from overactivation of the fibrinolytic system and bleeding, and whether or not you need heparin to treat their thrombus. You really don’t want to be doing this in your emergency department."
Hemorrhagic bullae also are a classic presentation of necrotizing fasciitis, along with pain out of proportion for typical cellulitis, systemic toxicity, crepitus, and rapid spread along fascial planes. Not all cases of necrotizing fasciitis will have hemorrhagic bullae, but worry if you see this, she said.
Treat with strategic debridement and broad-spectrum antibiotics. Studies have shown that adding hyperbaric oxygen therapy decreases mortality risk. It’s not worth transferring someone with necrotizing fasciitis to someplace hours away in order to get hyperbaric oxygen, but if this is available, "please use it," she urged. In the literature, mortality rates with necrotizing fasciitis range from 0% to 75%. "Guess which ones had 0% mortality patients who got hyperbaric oxygen."
Consider steroids for bullous rash with mucosal involvement. A woman came to Dr. Murphy-Lavoie’s emergency department complaining of a rash and pain with swallowing. She appeared moderately toxic, was tachycardic, had a bullous rash, and had dry mucous membranes but with oral lesions. Diagnosis: pemphigus vulgaris, which will involve mucosal surfaces 70% of the time.
The bullae may coalesce, and there may be a positive Nikolsky sign (sloughing of full-thickness skin with lateral pressure) and a positive Asboe-Hansen sign (light lateral pressure on the blister edge spreads the blister into adjacent clinically normal skin).
The biggest favor that emergency physicians can do for these patients is to start them on steroids. Before the advent of steroid therapy for pemphigus vulgaris, 50%-90% of patients died, compared with 4%-15% who are treated with steroids. Also provide local wound care to prevent secondary infection. Pemphigus vulgaris is associated with autoimmune diseases, and long-term management with immunosuppressive drugs probably will be handled by a rheumatologist, she said.
Dr. Murphy-Lavoie reported having no financial disclosures. She codeveloped the free app "EM Rashes."