PORTLAND, Ore. - Cyclosporine is often the best rescue therapy for acute generalized pustular psoriasis in children, according to Dr. Kelly M. Cordoro.

“When speed is important, my first choice is cyclosporine,” said Dr. Cordoro of the department of dermatology at the University of California, San Francisco. When adequately dosed, it can end pustulation within days.

Dr. Kelly M. Cordoro

Methotrexate and acitretin are also options, but can take several weeks to have maximum benefit, which is too slow for acute generalized pustular psoriasis (GPP) patients. GPP can cause fatal secondary infections, calcium imbalances, and cardiorespiratory collapse, although these outcomes are rare in children. She also noted methotrexate’s hepatotoxic potential, a concern in the treatment of GPP because the disease itself can cause acute liver dysfunction.

Acute GPP patients, apart from the pathognomonic skin findings, can present with fever, anorexia, and chills. Patients “are very sick,” she said.

Severe cases warrant hospital admission for treatment and supportive care, including rehydration, electrolyte imbalance correction, and bed rest. Compresses followed by bland emollients, such as petrolatum jelly, are essential to help prevent secondary infections and sepsis.

Rescue therapy is only the first of three stages by which Dr. Cordoro conceptualizes GPP management.

The second, transition therapy, usually includes tapering the cyclosporine and initiating other treatments for long-term control, such as acitretin, phototherapy, or both.

The maintenance stage comes after the patient has been stable or clear for months. Phototherapy and topical vitamin D analogues, such as Dovonex, are “great choices for maintenance,” Dr. Cordoro said. Low-dose acitretin, topical corticosteroids, and topical calcineurin inhibitors are other options.

Photo courtesy Dr. Kelly Cordoro. An 8 year-old boy with an acute flare of generalized pustular psoriasis.

Dr. Cordoro also tests the pharynx and perianal area for group A streptococcus colonization, and treats to eliminate it once her GPP patients are well enough for antibiotics. “It’s not known if it helps in the acute setting, but strep antigen may trigger psoriasis again at some point,” she said.

There are no consensus treatment guidelines for GPP in children; clinical decisions are based on patient characteristics, clinical experience and setting, and hunches about the best way to proceed. “There’s no cookbook approach, no one-size-fits-all treatment,” Dr. Cordoro said.

One of her patients, an 8-year-old boy with severe, relapsing/remitting GPP, illustrates the point.

He had been doing fairly well on low-dose acitretin, but then flared. His mother took him to an urgent care center, and the doctor there, mistaking GPP for an infection, started the boy on a cephalosporin.

His condition worsened and he was toxic by the time Dr. Cordoro arrived.

She admitted the patient to the hospital and initiated rescue doses of cyclosporine 5 mg/kg per day, along with supportive care. His condition deteriorated over the next few days, and his liver enzymes became elevated. Since he was failing on cyclosporine, she wanted to use infliximab for rescue therapy.

09/17/10