Clinicians report what is believed to be the first US case of successfully treating life-threatening generalized pustular psoriasis (GPP) of pregnancy with the IL-36 receptor antagonist spesolimab, suggesting a promising new therapeutic option for this high-risk condition.
In the report, published in Pregnancy, authors led by Jason Bunn, MD, of the division of maternal-fetal medicine in the department of obstetrics and gynecology at the University of Oklahoma Medical Center, described the clinical course of a 39-year-old multiparous African American woman who presented at 19 weeks’ gestation with rapidly progressive GPP. Her medical history included chronic hypertension, obesity, a prior classical cesarean, spontaneous preterm birth, and previous preeclampsia, all of which placed her at elevated obstetric risk. The onset of a diffuse, scaling eruption that progressed to blistering and desquamation within days quickly escalated her condition into a dermatologic and obstetric emergency.
Despite aggressive management, including high-dose systemic corticosteroids, cyclosporine, and two infliximab infusions, her disease continued to deteriorate. She required transfer to a burn intensive care unit for pain control, hydration, and close monitoring. Laboratory evaluation revealed leukocytosis and electrolyte abnormalities, while biopsy confirmed pustular psoriasis of pregnancy with intraepidermal neutrophilic pustules. Mucous membranes were spared.
Prolonged corticosteroid exposure soon exacerbated her hypertension and triggered significant hyperglycemia that required high-dose insulin therapy. Her clinicians noted increasing concerns about the risks posed by ongoing disease activity and by continued reliance on glucocorticoids, traditionally the mainstay of GPP treatment but associated with adverse maternal–fetal effects.
After extensive counseling and reviewing available safety data, the team administered intravenous spesolimab, a monoclonal antibody approved in 2022 specifically for GPP despite a paucity of pregnancy data. The patient experienced rapid clinical improvement, with marked resolution of pustules within one week. She was discharged at 27 weeks’ gestation on a tapering steroid regimen and transitioned to subcutaneous spesolimab every four weeks, maintaining response without flares. Serial ultrasounds demonstrated normal fetal growth and anatomy.
At 37 weeks, she underwent an uncomplicated scheduled repeat cesarean delivery. She delivered a healthy female neonate weighing 2765 g with normal Apgar scores, and no neonatal complications were reported. Maternal postpartum recovery was notable only for pain management challenges, transient insulin requirements related to perioperative steroid dosing, and later identification of a small abdominal wall hernia. Dermatologic follow-up showed minimal residual xerosis and no active pustular disease.
The authors noted that GPP of pregnancy, historically termed impetigo herpetiformis, remains a rare but high-risk dermatosis associated with septic complications, placental insufficiency, and stillbirth. “Signs and symptoms include fatigue, malaise, leukocytosis and sterile pustules in intertriginous areas that spread and desquamate centrifugally,” they wrote. “Maternal mortality may be as high as 16% as a consequence of septic shock or cardiopulmonary failure.”
They stated that only one other GPP of pregnancy case, treated in China, has been published to date, and concluded that further research is needed to determine whether IL-36R antagonists could form a new therapeutic class capable of rapidly and safely resolving serious immunologic disease in pregnancy.
The authors reported having no disclosures.
Source: Pregnancy