A Life-Saving Treatment in a 60-year Old Patient With Rapid Eruption of Pustules
Case author Boni Elewski, MD and moderator Danny Zakria, MD, MBA, present a case of a 60-year old female who was airlifted in the middle-of-the-night with a rare dermatologic emergency.
By Boni Elewski, MD
This case describes a 60-year-old Caucasian female with a past medical history of plaque psoriasis and psoriatic arthritis well-controlled on guselkumab 100mg every 8 weeks who presented to the emergency department with an acute generalized pustular dermatosis. This rash developed two days after she received intramuscular triamcinolone and oral methylprednisolone to treat a presumed morbilliform drug eruption that appeared less than 1 week after she took oral clindamycin for paronychia. Of note, she was diagnosed with psoriasis as a teenager and had been taking guselkumab for about 2 years.
She was life-flighted to the University of Alabama at Birmingham in the middle of the night due to concern over the severity of her illness. On presentation, she had a fever of 103.9, significant chills, tacyhcardia, and a leukocytosis requiring admission to the intensive care unit with a Dermatology consult.
Physical Exam, Lab Results and Medical History
Physical exam demonstrated coalescing and isolated pustules within a background of erythema involving the face, neck, trunk, groin, upper extremities, and lower extremities. There was substantial edema of the face, trunk, and extremities. The dermatology team performed two 4mm punch biopsies of the left abdomen and left thigh. These biopsies revealed subcorneal and intraepidermal spongiform pustules consistent with pustular psoriasis.
Diagnostic Review
The differential diagnosis for generalized pustular psoriasis (GPP) includes acute generalized exanthematous pustulosis (AGEP), subcorneal pustular dermatosis, subacute annular pustular psoriasis (APP), IgA pemphigus, Sneddon Wilkerson disease, and pemphigus foliaceus. It is particularly important to exclude AGEP, another rare and severe pustular skin eruption. AGEP typically has a more abrupt onset and shorter duration and is not associated with a personal or family history of psoriasis.1
GPP is often challenging to treat due its unpredictable clinical course that is characterized by persistent disease or intermittent relapsing flares. Additionally, many of the classic treatment options have variable efficacy and are associated with several adverse effects.
Based on the patient's clinical presentation, which of the following would be highest on your differential diagnosis?
Before & After Photos
Treatment Discussion and Outcome
This patient was treated with a single intravenous infusion of spesolimab 900mg along with triamcinolone 0.1% ointment and hydrocortisone 1% cream. She had very rapid and impressive improvement in her pustular eruption within 18 hours of receiving spesolimab, as shown in the images above. After 48 hours she demonstrated continued improvement with complete resolution of all remaining pustules. She tolerated the infusion well without any reported adverse effects.
Spesolimab is the first FDA-approved treatment for GPP in adults and works by antagonizing the IL-36 receptor, thus inhibiting this cytokine's proinflammatory cascades.2 Dosing of spesolimab for the treatment of GPP involves one 900mg intravenous infusion followed by an additional 900mg infusion one week later if the flare persists. Adverse effects noted in one randomized controlled trial studying spesolimab for GPP included pyrexia, drug reaction, infection, and hepatic injury.4 However, no patients had to discontinue treatment. Compared to other therapies for GPP such as cyclosporine or methotrexate, spesolimab may be much more tolerable and efficacious.
The first FDA-approved treatment for generalized pustular psoriasis in adults targets which cytokine?
Case Discussion
Mark Lebwohl, MD Professor and Chairman Department of Dermatology Icahn School of Medicine at Mount Sinai