New Onset Generalized Pustular Psoriasis Rapidly Improved with IL-36 Blockade

Main Article Content

Meredith Burns
Timothy Orlowski
Hoang Ho-Pham
Carly Elston
Boni Elewski

Keywords

Generalized pustular psoriasis, Interleukin-36 (IL-36) receptor antagonist, Spesolimab

Abstract

Generalized pustular psoriasis is a relatively rare variant of psoriasis characterized by diffuse eruption of sterile pustules on an erythematous background. Untreated flares can result in life-threatening complications including sepsis and organ failure. Generalized pustular psoriasis has historically proven challenging to treat with an unpredictable clinical course and paucity of reliably efficacious treatment options. Until recently, there have been no FDA-approved therapies for generalized pustular psoriasis in the United States. In September 2022, the IL-36 receptor antagonist spesolimab became the first FDA-approved treatment for generalized pustular psoriasis in adults. We present a case of generalized pustular psoriasis with complete and rapid response to spesolimab.

References

1. Choon SE, Lai NM, Mohammad NA, Nanu NM, Tey KE, Chew SF. Clinical profile, morbidity, and outcome of adult-onset generalized pustular psoriasis: analysis of 102 cases seen in a tertiary hospital in Johor, Malaysia. Int J Dermatol. 2014;53(6):676-684.

2. 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.

3. Benjegerdes KE, Hyde K, Kivelevitch D, Mansouri B. Pustular psoriasis: pathophysiology and current treatment perspectives. Psoriasis (Auckl). 2016;6:131-144. Published 2016 Sep 12.

4. Lowe NJ, Ridgway HB. Generalized pustular psoriasis precipitated by lithium carbonate. Arch Dermatol. 1978;114(12):1788-1789.

5. Gooderham MJ, Van Voorhees AS, Lebwohl MG. An update on generalized pustular psoriasis. Expert Rev Clin Immunol. 2019;15(9):907-919.

6. Marrakchi S, Guigue P, Renshaw BR, et al. Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis. N Engl J Med. 2011;365(7):620-628.

7. Matarazzo L, Hernandez Santana YE, Walsh PT, Fallon PG. The IL-1 cytokine family as custodians of barrier immunity. Cytokine. 2022;154:155890.

8. Iznardo H, Puig L. IL-1 Family Cytokines in Inflammatory Dermatoses: Pathogenetic Role and Potential Therapeutic Implications. Int J Mol Sci. 2022;23(16):9479. Published 2022 Aug 22.

9. Robinson A, Van Voorhees AS, Hsu S, et al. Treatment of pustular psoriasis: from the Medical Board of the National Psoriasis Foundation. J Am Acad Dermatol. 2012;67(2):279-288.

10. Shao S, Wang G. Commentary on a Clinical Trial of Spesolimab, a Humanized Anti-interleukin-36 Receptor Monoclonal Antibody, in Generalized Pustular Psoriasis [published online ahead of print, 2022 Oct 8]. Dermatol Ther (Heidelb). 2022;10.1007/s13555-022-00830-x.

11. Bachelez H, Choon SE, Marrakchi S, et al. Inhibition of the Interleukin-36 Pathway for the Treatment of Generalized Pustular Psoriasis. N Engl J Med. 2019;380(10):981-983.

12. Bachelez H, Choon SE, Marrakchi S, et al. Trial of Spesolimab for Generalized Pustular Psoriasis. N Engl J Med. 2021;385(26):2431-2440.

Most read articles by the same author(s)