Medical mystery: The infection that would not stop

A 43-year-old woman came to a hospital complaining of abdominal pain. She was in poor health generally, with serious kidney disease, insulin-dependent diabetes, vascular disease, and obesity. She also had stomach ulcers, but the pain that brought her to the hospital was like nothing she'd ever experienced.

She had been taking antibiotics for a urinary tract infection and developed blisters, which suggested an allergic reaction.

Hospital staff quickly found her to be in severe septic shock. In addition to the urinary tract infection was a more serious drug-resistant infection in the lining of her stomach. This infection, or peritonitis, was likely the cause of her stomach pain.

She was admitted to the intensive care unit, but developed pneumonia and anemia so serious that she needed blood transfusions.

Clinicians searched to find out what was making their patient so sick and came up with a list that included toxic epidermal necrolysis (TEN), a life-threatening skin disease usually caused by an underlying infection or an adverse drug reaction. This condition typically includes blisters and massive skin peeling.

On Day 4, large, fluid-filled blisters appeared on her hands, abdomen, breasts, and back. At this stage, our Regional Burn Center, which specializes in soft-tissue diseases as well as burns, was contacted and received the transferred patient.

A biopsy of her right forearm confirmed a diagnosis of TEN. But unlike a classic TEN diagnosis, her eyes and mouth tissue were barely affected. And her ears, scalp, and head were spared from the peeling that had affected more than 50 percent of her body.

Despite additional antibiotics, ventilator support, doses of hydrocortisone, and multiple vasopressors - powerful drugs used to elevate blood pressure in critically ill patients - her condition continued to deteriorate. Something else was clearly going on in addition to TEN.

Within days, the woman's fingers and toes turned blue. A fungal superinfection was added to her diagnosis, and sepsis set in. Skin lesions developed. Blood flow to her limbs was lost.

In addition to TEN and the fungal superinfection, she was further diagnosed with a rare and devastating condition called purpura fulminans. It takes its name from its characteristic purpuric lesions, hemorrhages in the skin and mucous membranes that leave purplish patches.

We could do nothing to save her life. Her family chose to pursue comfort care, and the patient was allowed to die peacefully soon afterward.

Most cases of PF occur in children, though it can also affect adults who have suffered a serious infection.

Could this particular case have been prevented? It's hard to say. It's possible that a previous vaccination against flu or pneumonia might have protected her from an illness that weakened her ability to fight subsequent infections. More rapid treatment with different antibiotics and surgery, as well as a quicker identification of the underlying cause, might have helped. Still, PF usually is fatal, and this patient did have numerous health conditions that would have made it harder to fight such an enormous infection.

Our burn center cares for an adult patient with PF every two or three months. Recently we treated a young professional woman who survived, but had to have amputation of two of her limbs.

More research into the causes and treatment of PF are needed as the regional medical community works together to ensure that the signs and symptoms of PF are recognized as early as possible.