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Review
. 2020 Aug 14:13:561-578.
doi: 10.2147/CCID.S240607. eCollection 2020.

Diagnosis, Screening and Treatment of Patients with Palmoplantar Pustulosis (PPP): A Review of Current Practices and Recommendations

Egídio Freitas  1 , Maria Alexandra Rodrigues  1 , Tiago Torres  1   2
Affiliations
Review

Diagnosis, Screening and Treatment of Patients with Palmoplantar Pustulosis (PPP): A Review of Current Practices and Recommendations

Egídio Freitas et al. Clin Cosmet Investig Dermatol. .

Abstract

Palmoplantar pustulosis (PPP) is a rare, chronic, recurrent inflammatory disease that affects the palms and/or the soles with sterile, erupting pustules, which are debilitating and usually resistant to treatment. It has genetic, histopathologic and clinical features that are not present in psoriasis; thus, it can be classified as a variant of psoriasis or as a separate entity. Smoking and upper respiratory infections have been suggested as main triggers of PPP. PPP is a challenging disease to manage, and the treatment approach involves both topical and systemic therapies, as well as phototherapy and targeted molecules. No gold standard therapy has yet been identified, and none of the treatments are curative. In patients with mild disease, control may be achieved with on-demand occlusion of topical agents. In patients with moderate-to-severe PPP, phototherapy or a classical systemic agent (acitretin being the best treatment option, especially in combination with PUVA) may be effective. Refractory patients or those with contraindications to use these therapies may be good candidates for apremilast or biologic therapy, particularly anti-IL-17A and anti-IL-23 agents. Recent PPP trials are focusing on blockage of IL-36 or IL-1 pathways, which play an important role in innate immunity. Indeed, IL-36 isoforms have been strongly implicated in the pathogenesis of psoriasis. Therefore, blockage of the IL-36 pathway has become a new treatment target in PPP, and three studies are currently evaluating the use of monoclonal antibodies that block the IL-36 receptor in PPP: ANB019 and spesolimab (BI 655130). In this review, we explore the diagnosis, screening and treatment of patients with PPP.

Keywords: ANB019; IL36; palmoplantar pustulosis; palmoplantar pustulosis psoriasis; spesolimab.

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Conflict of interest statement

Egidio Freitas and Maria Alexandra Rodrigues have no conflicts of interest to declare. Tiago Torres has received research grants and/or consulting fees from AbbVie, Almirall, Amgen, Arena Pharmaceuticals, Biocad, Boehringer Ingelheim, Bristol-Myers Squibb, Celgene, Eli Lilly, Janssen, LEO Pharma, MSD, Novartis, Pfizer, Samsung-Bioepis, Sandoz, Sanofi.

Figures

Figure 1
Figure 1
Scattered pustules on a erythematous base with desquamation on the medial zone of the plantar surface of the foot.
Figure 2
Figure 2
Pustules, sometimes coalescing, with erythematous halo, affecting mainly the palms.
Figure 3
Figure 3
Pustular lesions in a erythematous and scaling plaque affecting the central zone of the plantar surface of the foot.
Figure 4
Figure 4
Erythema, multiple pustules and scaling in the palmar surface of the hand.
Figure 5
Figure 5
A closer look of Figure 4.
Figure 6
Figure 6
The pustular eruption affecting both palmar surfaces of the hands.
See this image and copyright information in PMC

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