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A 66-year-old woman presents with a flare of a chronic skin condition. Over the past 2 decades, the disorder had been treated with numerous therapies with some achieving adequate control for variable periods but each subsequently losing efficacy. One injectable medication resulted in approximately 90% clearance for approximately 18 months; however, her insurance coverage would no longer cover this drug and a substituted same-class agent proved ineffective after a 4-month trial (Figure). On physical examination, the patient is afebrile with extensive tender erythematous papules with some scaling and itching.
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Pustular psoriasis is one of the least common variants of psoriasis, occurring in approximately 1% of cases.1 Sterile pustules arising on an erythematous base characterize the condition and result from accumulation of neutrophils beneath the stratum corneum.2 Subtypes of pustular psoriasis are defined by the distribution of pustules. When pustules arise primarily on palms and soles, the condition is referred to as palmoplantar pustulosis. A variant involving the fingers, toes, and nails is termed acrodermatitis continua of Hallopeau.3 Pustules distributed over the body surface characterize generalized pustular psoriasis, also known as von Zumbusch psoriasis. Patients with generalized pustular psoriasis may experience fever, chills, anorexia, and systemic involvement. The condition can be life-threatening and require hospitalization.4
Pustular psoriasis is most frequently associated with a mutation of interleukin (IL)36RN gene.5 Other mutations associated with generalized pustular psoriasis involve AP1S3 and CARD14 genes. The untoward result is activation of proinflammatory cytokines including IL-36, which induce aberrant keratinocyte proliferation and accumulations of pustules.6
Treatment of pustular psoriasis is difficult. Generalized pustular psoriasis tends to be chronic with inconsistent response to systemic agents such as cyclosporine, acitretin, and methotrexate.7 Randomized controlled studies conducted with the biologic agents secukinumab and guselkumab did not demonstrate superiority over placebo. The condition is characterized by intermittent flares, which may spontaneously self-remit. An intravenous biologic that targets IL-36 (spesolimab) was approved in 2022 as treatment for generalized pustular psoriasis.8
Taylor Jones, OMS-II, is a medical student at the West Virginia School of Osteopathic Medicine in Lewisburg, West Virgina. Stephen Schleicher, MD, is director of the DermDox Dermatology Centers, associate professor of medicine at Geisinger Commonwealth Medical College, and clinical instructor of dermatology at Arcadia University and Kings College.
References
1. Marrakchi S, Puig L. Pathophysiology of generalized pustular psoriasis. Am J Clin Dermatol. 2022;23(Suppl 1):13-19. doi:10.1007/s40257-021-00655-y
2. Fujita H, Gooderham M, Romiti R. Diagnosis of generalized pustular psoriasis. Am J Clin Dermatol. 2022;23(Suppl 1):31-38. doi:10.1007/s40257-021-00652-1
3. Menter A, Van Voorhees AS, Hsu S. Pustular psoriasis: a narrative review of recent developments in pathophysiology and therapeutic options. Dermatol Ther (Heidelb). 2021;11(6):1917-1929. doi:10.1007/s13555-021-00612-x
4. Zheng M, Jullien D, Eyerich K. The prevalence and disease characteristics of generalized pustular psoriasis. Am J Clin Dermatol. 2022;23(Suppl 1):5-12. doi:10.1007/s40257-021-00664-x
5. Singh R, Koppu S, Perche PO, Feldman SR. The cytokine mediated molecular pathophysiology of psoriasis and its clinical implications. Int J Mol Sci. 2021;22(23):12793. doi:10.3390/ijms222312793
6. Genovese G, Moltrasio C, Cassano N, Maronese CA, Vena GA, Marzano AV. Pustular psoriasis: from pathophysiology to treatment. Biomedicines. 2021;9(12):1746. doi:10.3390/biomedicines9121746
7. Bachelez H. Pustular psoriasis: the dawn of a new era. Acta Derm Venereol. 2020;100(3):adv00034. doi:10.2340/00015555-3388
8. Ernst D. Spevigo approved to treat generalized pustular psoriasis flares. MPR. Accessed March 7, 2023. https://www.empr.com/home/news/spevigo-approved-to-treat-generalized-pustular-psoriasis-flares/
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