A brief guide to pustular psoriasis for primary care providers

ABSTRACT

Pustular psoriasis refers to a heterogeneous group of chronic inflammatory skin disorders that are clinically, histologically, and genetically distinct from plaque psoriasis. Pustular psoriasis may present as a recurrent systemic illness (generalized pustular psoriasis [GPP]), or as localized disease affecting the palms and/or soles (palmoplantar pustulosis [PPP], also known as palmoplantar pustular psoriasis), or the digits/nail beds (acrodermatitis continua of Hallopeau [ACH]). These conditions are rare, but their possible severity and consequences should not be underestimated. GPP, especially an acute episode (flare), may be a medical emergency, with potentially life-threatening complications. PPP and ACH are often debilitating conditions. PPP is associated with impaired health-related quality of life and psychiatric morbidity, while ACH threatens irreversible nail and/or bone damage. These conditions can be difficult to diagnose; thus, primary care providers should not hesitate to contact a dermatologist for advice and/or patient referral. The role of corticosteroids in triggering and leading to flares of GPP should also be noted, and physicians should avoid the use of systemic corticosteroids in the management of any form of psoriasis.

PLAIN LANGUAGE SUMMARY

A brief guide to pustular psoriasis for primary care providers

Pustular psoriasis consists of a group of rare conditions that cause the skin to become red and painful. In these conditions, small blisters filled with pus (called pustules) appear suddenly. The pustules are not infectious. Pustular psoriasis is different from plaque psoriasis, in which people develop scaly patches of skin. People can have pustular psoriasis and plaque psoriasis at the same time. Pustular psoriasis can be widespread, affecting large areas of the body, arms, and legs. This is called generalized pustular psoriasis (GPP). GPP can cause life-threatening complications that may require emergency medical treatment. Pustular psoriasis can be more localized, occurring on the palms of the hands and soles of the feet. This is called palmoplantar pustulosis (PPP). It can also occur on the fingers, toes, and nail beds, called acrodermatitis continua of Hallopeau (ACH). PPP and ACH can make walking and other everyday activities difficult. Because GPP, PPP, and ACH are rare, primary care providers are unlikely to meet many people with pustular psoriasis, so they may not recognize these conditions immediately. This article aims to help primary care providers assess and diagnose people who may have GPP, PPP, or ACH, and advise when they should get help from a skin specialist (dermatologist). See Figure 1 for a full infographic version of this summary.

KEYWORDS:

• Acrodermatitis continua of Hallopeau
• generalized pustular psoriasis
• palmoplantar pustulosis
• primary care
• psoriasis
• von Zumbusch

Acknowledgments

Medical writing assistance, supported financially by Boehringer Ingelheim Pharmaceuticals, Inc. (BIPI), was provided by Debra Brocksmith, MB ChB, PhD, of Elevate Scientific Solutions during the preparation of this manuscript. BIPI was given the opportunity to check the data used in this manuscript for factual accuracy only.

Contribution statement

All authors contributed to the conceptualization, critical review, revision, and approval of the final version of this manuscript.

Data sharing

Data sharing is not applicable to this article, as no new data were created or analyzed in this report.

Declaration of interest

No potential conflict of interest was reported by the authors.

Declaration of financial/other relationships

JJC: Acted as a consultant/speaker/investigator for Abbvie, Lilly, Novartis, Janssen, Regeneron, Sanofi, Sun Pharma, and UCB; acted as a consultant/investigator for Dermira; acted as a consultant for Boehringer Ingelheim; acted as an investigator for Merck, Pfizer, Sandoz, MC2 Therapeutics, and Verrica Pharmaceuticals.

DMP: Acted as a consultant for Atacama Therapeutics, Bickel Biotechnology, Biofrontera AG, BMS, Celgene Corporation, Dermira, LEO Pharma US, Novartis Pharmaceuticals Corp., Pfizer Inc., Regeneron, Sanofi, TDM SurgiTech, Inc., TheraVida, and Valeant Pharmaceuticals International; and received grant funding from Abbott Laboratories, Almirall, Amgen, AOBiome LLC, Asana Biosciences LLC, Bickel Biotechnology, Celgene Corporation, Dermavant Sciences, Dermira, Eli Lilly and Company, LEO Pharma US, Menlo Therapeutics, Merck & Co., Inc., Novartis Pharmaceuticals Corp., Novo Nordisk A/S, Ortho Dermatologics, Pfizer Inc., Regeneron, Stiefel (a GlaxoSmithKline company), and Valeant Pharmaceuticals International.

PSY: Speaker, consultant, investigator for Abbvie, Amgen, Celgene, Janssen, LEO, Lilly, Novartis, Ortho Dermatologics, Pfizer, Sun Pharma, UCB; Consultant and investigator for Arcutis, BMS, Boehringer Ingelheim, Dermavant, Anaptys Bio.

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This work was supported by Boehringer Ingelheim Pharmaceuticals, Inc., Ridgefield, CT, USA. The authors received no direct compensation related to the development of the manuscript.