Palmoplantar pustular psoriasis: an update on pathophysiology and review of treatment options
Abstract
Palmoplantar pustular psoriasis (PPPP) is a localized variant of pustular psoriasis that does not respond predictably to conventional psoriasis treatments and is a notoriously challenging condition to manage even though it shares many clinical and histopathologic features with plaque psoriasis. This review will briefly explore the clinical and pathophysiological features of PPPP and look at the evidence behind treatment of this condition using systemic and advanced therapies.
References
Yamamoto T. Similarity and difference between palmoplantar pustulosis and pustular psoriasis. J Dermatol. 2021;48(6):750-760. doi:10.1111/1346-8138.15826
Bissonnette R, Suárez-Fariñas M, Li X, et al. Based on Molecular Profiling of Gene Expression, Palmoplantar Pustulosis and Palmoplantar Pustular Psoriasis Are Highly Related Diseases that Appear to Be Distinct from Psoriasis Vulgaris. PLOS ONE. 2016;11(5):e0155215. doi:10.1371/journal.pone.0155215
Uppala R, Tsoi LC, Harms PW, et al. “Autoinflammatory psoriasis”-genetics and biology of pustular psoriasis. Cell Mol Immunol. 2021;18(2):307-317. doi:10.1038/s41423-020-0519-3
Akiyama T, Seishima M, Watanabe H, Nakatani A, Mori S, Kitajima Y. The relationships of onset and exacerbation of pustulosis palmaris et plantaris to smoking and focal infections. J Dermatol. 1995;22(12):930-934. doi:10.1111/j.1346-8138.1995.tb03948.x
Eriksson MO, Hagforsen E, Lundin IP, Michaëlsson G. Palmoplantar pustulosis: a clinical and immunohistological study. Br J Dermatol. 1998;138(3):390-398. doi:10.1046/j.1365-2133.1998.02113.x
Brunasso AMG, Puntoni M, Aberer W, Delfino C, Fancelli L, Massone C. Clinical and epidemiological comparison of patients affected by palmoplantar plaque psoriasis and palmoplantar pustulosis: a case series study. Br J Dermatol. 2013;168(6):1243-1251. doi:10.1111/bjd.12223
Ferreli C, Pinna AL, Pilloni L, Tomasini CF, Rongioletti F. Histopathological aspects of psoriasis and its uncommon variants. G Ital Dermatol E Venereol Organo Uff Soc Ital Dermatol E Sifilogr. 2018;153(2):173-184. doi:10.23736/S0392-0488.17.05839-4
Murakami M, Terui T. Palmoplantar pustulosis: Current understanding of disease definition and pathomechanism. J Dermatol Sci. 2020;98(1):13-19. doi:10.1016/j.jdermsci.2020.03.003
Bissonnette R, Nigen S, Langley RG, et al. Increased expression of IL-17A and limited involvement of IL-23 in patients with palmo-plantar (PP) pustular psoriasis or PP pustulosis; results from a randomised controlled trial. J Eur Acad Dermatol Venereol JEADV. 2014;28(10):1298-1305. doi:10.1111/jdv.12272
Murakami M, Kaneko T, Nakatsuji T, et al. Vesicular LL-37 contributes to inflammation of the lesional skin of palmoplantar pustulosis. PloS One. 2014;9(10):e110677. doi:10.1371/journal.pone.0110677
Hagforsen E, Hedstrand H, Nyberg F, Michaëlsson G. Novel findings of Langerhans cells and interleukin-17 expression in relation to the acrosyringium and pustule in palmoplantar pustulosis. Br J Dermatol. 2010;163(3):572-579. doi:10.1111/j.1365-2133.2010.09819.x
Baum P, Visvanathan S, Garcet S, et al. Pustular psoriasis: Molecular pathways and effects of spesolimab in generalized pustular psoriasis. J Allergy Clin Immunol. 2022;149(4):1402-1412. doi:10.1016/j.jaci.2021.09.035
Twelves S, Mostafa A, Dand N, et al. Clinical and genetic differences between pustular psoriasis subtypes. J Allergy Clin Immunol. 2019;143(3):1021-1026. doi:10.1016/j.jaci.2018.06.038
Murakami M, Hagforsen E, Morhenn V, Ishida-Yamamoto A, Iizuka H. Patients with palmoplantar pustulosis have increased IL-17 and IL-22 levels both in the lesion and serum. Exp Dermatol. 2011;20(10):845-847. doi:10.1111/j.1600-0625.2011.01325.x
Hagforsen E, Edvinsson M, Nordlind K, Michaëlsson G. Expression of nicotinic receptors in the skin of patients with palmoplantar pustulosis. Br J Dermatol. 2002;146(3):383-391. doi:10.1046/j.1365-2133.2002.04640.x
Michaëlsson G, Gustafsson K, Hagforsen E. The psoriasis variant palmoplantar pustulosis can be improved after cessation of smoking. J Am Acad Dermatol. 2006;54(4):737-738. doi:10.1016/j.jaad.2005.07.024
Lu JD, Lytvyn Y, Mufti A, et al. Biologic therapies associated with development of palmoplantar pustulosis and palmoplantar pustular psoriasis: a systematic review. Int J Dermatol. Published online February 6, 2022. doi:10.1111/ijd.16064
Conrad C, Di Domizio J, Mylonas A, et al. TNF blockade induces a dysregulated type I interferon response without autoimmunity in paradoxical psoriasis. Nat Commun. 2018;9:25. doi:10.1038/s41467-017-02466-4
Benjegerdes KE, Hyde K, Kivelevitch D, Mansouri B. Pustular psoriasis: pathophysiology and current treatment perspectives. Psoriasis Auckl NZ. 2016;6:131-144. doi:10.2147/PTT.S98954
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. doi:10.1016/j.jaad.2011.01.032
Reich K, Graff O, Mehta N. Oral alitretinoin treatment in patients with palmoplantar pustulosis inadequately responding to standard topical treatment: a randomized phase II study. Br J Dermatol. 2016;174(6):1277-1281. doi:10.1111/bjd.14401
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
Menter A, Van Voorhees AS, Hsu S. Pustular Psoriasis: A Narrative Review of Recent Developments in Pathophysiology and Therapeutic Options. Dermatol Ther. 2021;11(6):1917-1929. doi:10.1007/s13555-021-00612-x
Misiak-Galazka M, Zozula J, Rudnicka L. Palmoplantar Pustulosis: Recent Advances in Etiopathogenesis and Emerging Treatments. Am J Clin Dermatol. 2020;21(3):355-370. doi:10.1007/s40257-020-00503-5
Sanchez IM, Sorenson E, Levin E, Liao W. The Efficacy of Biologic Therapy for the Management of Palmoplantar Psoriasis and Palmoplantar Pustulosis: A Systematic Review. Dermatol Ther. 2017;7(4):425-446. doi:10.1007/s13555-017-0207-0
Richetta AG, Mattozzi C, Giancristoforo S, et al. Safety and efficacy of Adalimumab in the treatment of moderate to severe palmo-plantar psoriasis: an open label study. Clin Ter. 2012;163(2):e61-66.
Bissonnette R, Poulin Y, Bolduc C, et al. Etanercept in the treatment of palmoplantar pustulosis. J Drugs Dermatol JDD. 2008;7(10):940-946.
Morales-Múnera C, Vilarrasa E, Puig L. Efficacy of ustekinumab in refractory palmoplantar pustular psoriasis. Br J Dermatol. 2013;168(4):820-824. doi:10.1111/bjd.12150
Terui T, Kobayashi S, Okubo Y, et al. Efficacy and Safety of Guselkumab in Japanese Patients With Palmoplantar Pustulosis: A Phase 3 Randomized Clinical Trial. JAMA Dermatol. 2019;155(10):1153-1161. doi:10.1001/jamadermatol.2019.1394
Hugo J, Arenbergerova M, Arenberger P, Gkalpakiotis S. Coexistence of palmoplantar pustulosis and ichthyosis vulgaris treated by risankizumab. Br J Dermatol. 2021;184(6):e195. doi:10.1111/bjd.19835
Watabe D, Amano H. Successful treatment of adalimumab-induced paradoxical psoriatic arthritis with risankizumab. Eur J Dermatol EJD. 2020;30(5):611-612. doi:10.1684/ejd.2020.3859
Mrowietz U, Bachelez H, Burden AD, et al. Efficacy and safety of secukinumab in moderate to severe palmoplantar pustular psoriasis over 148 weeks: Extension of the 2PRECISE study. J Am Acad Dermatol. 2021;84(2):552-554. doi:10.1016/j.jaad.2020.06.038
Pinter A, Wilsmann-Theis D, Peitsch WK, Mössner R. Interleukin-17 receptor A blockade with brodalumab in palmoplantar pustular psoriasis: Report on four cases. J Dermatol. 2019;46(5):426-430. doi:10.1111/1346-8138.14815
Bachelez H, Choon SE, Marrakchi S, et al. Trial of Spesolimab for Generalized Pustular Psoriasis. N Engl J Med. 2021;385(26):2431-2440. doi:10.1056/NEJMoa2111563
Maçães CO, Lé AM, Torres T. Generalized pustular psoriasis: the new era of treatment with IL-36 receptor inhibitors. J Dermatol Treat. Published online June 21, 2022:1-8. doi:10.1080/09546634.2022.2089335
Mrowietz U, Burden AD, Pinter A, et al. Spesolimab, an Anti-Interleukin-36 Receptor Antibody, in Patients with Palmoplantar Pustulosis: Results of a Phase IIa, Multicenter, Double-Blind, Randomized, Placebo-Controlled Pilot Study. Dermatol Ther. 2021;11(2):571-585. doi:10.1007/s13555-021-00504-0
Wilsmann-Theis D, Kromer C, Gerdes S, et al. A multicentre open-label study of apremilast in palmoplantar pustulosis (APLANTUS). J Eur Acad Dermatol Venereol. 2021;35(10):2045-2050. doi:10.1111/jdv.17441
Personal Communication.
Li, C., Li, Z., Cao, Y., Li, L., Li, F., Li, Y., Xiong, D., Wu, X., Zhang, W., Zeng, X., 2021. Tofacitinib for the Treatment of Nail Lesions and Palmoplantar Pustulosis in Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis Syndrome. JAMA Dermatology 157, 74.. doi:10.1001/jamadermatol.2020.3095
Wang, Y.A., Rosenbach, M., 2020. Successful treatment of refractory tumor necrosis factor inhibitor-induced palmoplantar pustulosis with tofacitinib: Report of case. JAAD Case Reports 6, 115–118.. doi:10.1016/j.jdcr.2019.12.006
