By Gary M. White, MD
Inflammatory red plaques studded with pustules.
Generalized pustular psoriasis (GPP) is a severe, potentially life-threatening inflammatory dermatosis characterized by diffuse erythema and pustules. Fever, generalized erythema, and pustules develop acutely. Adults are typical, but infants and children may be affected. Tar may precipitate or exacerbate GPP and should be avoided. A geographic or fissured tongue may occur. Blood cultures may be indicated to exclude infection. See also psoriasis.
Patients with GPP and a subset of patients with AGEP have been found to have a mutation in the interleukin-36 receptor antagonist (IL-36Ra) [NEJM 2011;18:620–628]. Patients with pustular psoriasis including acrodermatitis continua of Hallopeau are at increased risk for AGEP [Andrew's Diseases of the Skin. Clinical Dermatology 2006]. Sometimes, treatment with antibiotics, particularly cephalexins can precipitate AGEP [J Dermatol Case Rep 2014:8:42-45] and their use should be avoided if possible.
Intraoral pustules have been proposed as a sign of IL-36Ra mutation [JAMA Derm 2015;151;452].
Erythematous plaques with pustules at the periphery are characteristic.
Hypoalbuminemia, hypocalcemia, loss of intravascular fluid, acute respiratory distress syndrome (ARDS), and renal failure are potential complications. Patients with ARDS present with shortness of breath and some of these patients have died. Leukopenia and leukocytosis occur.
A strep throat infection may trigger the disease or precipitate a flare. Pregnancy as well as various drugs have been reported to precipitate GPP including calcipotriene, tar, beta-blockers, lithium, and steroids. Generalized pustular psoriasis has developed in patients following withdrawal of cyclosporin for treatment of PPP.
The pus should be cultured and proven sterile. Ustekinumab alone or with acitretin completely controlled or cleared 4 patients with treatment-resistant PP [JAMADerm 2016;152;825]. This was independent of IL-36Ra status. Some of these patients had been initially controlled with etanercept and/or adalimumab.
Secukinumab, an anti-IL-17A antibody, improved one patient within 48 hours of the first dose with complete absence of pustulation within 7 days [JAMA Derm 2016;152;482]. In an open-label study of ixekizumab, another anti-IL-17A antibody, 5 patients with pustular psoriasis showed rapid and significant improvement [JEADV 2015;29;1148].
Anakinra, an interleukin (IL)-1 receptor antagonist, has been described as an effective treatment against GPP in several cases [British J Dermatology Jan 2014].
The new agent, gevokizumab, a monoclonal antibody against IL-1β, was used with great benefit in two patients with severe, recalcitrant GPP [BJD 2015;173;239].
A 38-year-old woman with a mutation in IL-36Ra and severe disease since age 6 was finally controlled nicely with adalimumab [BJD 2016;174;417]. Of note, during pregnancy, she had flares mimicking impetigo herpetiformis.
In the past, before the advent of biologics, acitretin 0.75 mg/kg/day (or isotretinoin, e.g., 1-1.5 mg/kg/day) was used initially to control the pustulation, followed by methotrexate and/or cyclosporin, e.g., 5-6 mg/kg/day.
Patient comfort should be addressed as these patients can lose heat, have painful skin lesions, and be relatively uncomfortable.
Later, the skin may come off in sheets of desquamation.
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