In their session on the first day of Fall Clinical 2022, “Working Together to Diagnose and Manage Patients with Generalized Pustular Psoriasis,” Boni E. Elewski, MD, and Alice B. Gottlieb, MD, PhD, discussed the prevalence and burden of generalized pustular psoriasis (GPP), diagnosing the condition, the current and evolving treatment options, and multidisciplinary team strategies. They were joined by Jordan Hicks, a patient speaker for the National Psoriasis Foundation, who shared her emotional journey through pustular psoriasis.

Dr Gottlieb started by presenting an overview of pustular psoriasis – a group of inflammatory skin diseases. In addition to generalized pustular psoriasis (GPP), subtypes include impetigo herpetiformis (pustular psoriasis of pregnancy), palmoplantar pustulosis, and annular pustular psoriasis.

She emphasized that GPP can be life-threatening, with patients appearing septic and needing emergency care. Pain is the predominant symptom of GPP, along with widespread sterile pustules and red, inflamed skin. Patients may present with malaise, fever, or tachycardia, among other signs and symptoms. GPP is rare, and emergency personnel may start the patient on I.V. antibiotics assuming they have sepsis. It mostly affects individuals in the middle-aged years and is more predominant in Asian patients. The mortality rate can be as high as 25%.

GPP has a genetic etiology, with patients in the IL-36 and CARD14 pathways predisposed to it. Environmental influences are important, such as an infection or the sudden withdrawal of systemic corticosteroids. It differs from plaque-type psoriasis because the main actor is more the early immune system and it is predominantly neutrophilic, driven by the IL-36 pathway. In contrast, the adaptive immune response plays a role in plaque psoriasis, which is driven by the IL-23 pathway.

When diagnosing GPP, the most important components are the history and physical examination. Do not just ask about the skin because patients can have cardiac compromise, or they can be experiencing depression. Usually there will be a relationship to a drug. Patients may be dehydrated. They often have a high white blood cell count, although there is no blood test that can confirm GPP. Patients may also be hypocalcemic or anemic, with high C-reactive protein and erythrocyte sedimentation rate. Pustules can be cultured if secondary infection is suspected. The most common differential diagnoses are acute generalized exanthematous pustulosis and dermatitis with secondary infection.

Patient speaker Jordan Hicks then told her story of recovering from pustular psoriasis. In 2021, she was experiencing chronic fatigue and what appeared to be spontaneous third-degree burns on her skin. She was prescribed oral steroids, which made her skin angrier. After being told that she was “wasting the doctor’s time” at an urgent care clinic, she saw a general practitioner who assumed she had a rash. Several months later, she saw a dermatologist and was diagnosed with palmoplantar pustulosis. She then saw a rheumatologist and was diagnosed with lupus and psoriatic arthritis. Her condition has responded to treatment, and she is monitored monthly by her rheumatologist. However, it has affected every aspect of her life. Ms. Hicks stated, “I was called hysterical…The ER saw me as a drug addict. My dermatologist saw me as dim-witted. My general practitioner saw me as a liar.” Dr Gottlieb reacted, “I think often when doctors don’t know what a patient has, they blame the patient, and that is really not fair.”

Dr Elewski rounded out the session with a discussion of managing pustular psoriasis and how clinicians can support patients. Due to systemic illness, patients may need hospitalization. It is important to identify drugs that may be inducing the flare and discontinue them. Initial therapeutic approaches, including biologics, depend on disease severity and drug availability. Extracutaneous complications, such as organ dysfunction, heart failure, hypocalcemia, and anemia must also be managed. Ms. Hicks commented, “Brain fog is a massive factor in this.” She continued, “It would be very nice to see doctors becoming more knowledgeable and being part of getting financial assistance. The government still sees psoriasis as dermatitis, so having a professional reaffirm that this is a systemic, horrible disease really makes an impact.”

Reference

Elewski BE, Gottlieb AB. Working together to diagnose and manage patients with generalized pustular psoriasis. Presented at: Fall Clinical Dermatology Conference 2022; October 20–23, 2022; Las Vegas, NV.