Palmoplantar Pustulosis

Disease Overview

Palmoplantar pustulosis (PPP) is a rare, recurrent inflammatory disorder. Affected individuals develop small to large sterile blisters filled with a yellow turbid liquid (pustules) on the palms of the hands and/or soles of the feet. The pustules may be painful and cause a burning feeling. The condition primarily affects females between 40 and 69 years of age, and mostly smokers.

The exact cause of PPP is not known. Researchers have determined that possible causes include smoking, bacterial infections (acute or chronic tonsillitis, dental infection, chronic sinusitis, contact allergies (mainly metals) and certain medications. Genetics may play a role and some patients have a family history of the disorder. PPP is strongly related to psoriasis vulgaris. Some patients have both PPP and plaque psoriasis or PPP and psoriatic arthritis. Psoriatic nail involvement is frequently seen in PPP and a family history of psoriasis has been reported in 10-42% of people with PPP.

Depending on the cause, there are many treatments available including certain moisturizers, medications (topical steroids and/or retinoids), light therapy and systemic therapies for severe cases with retinoids, conventional immunosuppressants (methotrexate, cyclosporine) and/or biological therapies.

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Synonyms

• pustulosis palmaris et plantaris
• PPP
• localized pustular psoriasis (LPP)
• palmoplantar pustular psoriasis

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Signs & Symptoms

People with palmoplantar pustulosis (PPP) may have the following signs and symptoms:

• Recurrent crops of sterile pustules on the palms of the hands and/or the soles of the feet
  • The pustules often merge on a red, inflamed background
  • Over several days these pustules can form scaly plaques with painful cracks
• Itching
• Pain
• Burning sensation in hand
• Difficulty walking and performing daily tasks with the hands and feet (due to the symptoms mentioned above).

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Causes

The exact cause of palmoplantar pustulosis (PPP) is not known. Researchers have found some possible causes including smoking, infections, certain medications and genetics.

• Smoking: Many patients who have PPP are smokers or have smoked in the past. Smoking may cause sweat glands to become inflamed, especially on the hands and feet, which causes pustules to form.
• Infections: Bacterial infections such as tonsillitis, chronic sinusitis and dental (odontogenic) infections have been associated with PPP onset and resolution of the infection is followed by skin improvement.
• Medications: Biological therapies, mainly anti-TNF-alpha molecules used to treat chronic inflammatory conditions such as psoriasis, rheumatoid arthritis, inflammatory bowel disease and others, have been associated with the onset of PPP due to the activation of the innate immune system.
• Contact allergies: Some researchers (mainly in Japan) have found that patients with PPP are allergic to nickel, chromium, mercury and fragrances (rare) and if the metal allergen is withdrawn the PPP might improve.
• Family history: People who have a family history of PPP may be more at risk of developing the condition, as well as patients with family history of psoriasis.

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Affected populations

Based on U.S. data, the estimated prevalence of PPP in 2020 was ≤ 1.28 in 100,000 population among patients ≤ 11 years of age and ≤ 2.37 among patients 12–17 years of age. PPP mostly affects females between 40 and 69 years of age and smokers, but this condition may affect people of any age.

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Disorders with Similar Symptoms

Some researchers suggest that PPP is a type of psoriasis, while others suggest that it is a separate disorder. Psoriasis is a skin condition that causes skin cells to multiply too quickly after inflammation. Symptoms of psoriasis include itchy, scaly skin and dry, red patches which may be painful.

Rarely PPP can be part of a syndrome called SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) an autoinflammatory neutrophilic disease most frequently seen in Japan.

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Diagnosis

A doctor will look at the affected skin and skin swabs might be indicated to confirm the sterile nature (without bacteria or fungi) inside the pustules. In some patients, a skin biopsy will be needed. If a biopsy is necessary, the doctor will take a piece of skin from the affected area and look under a microscope for features of the disease.

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Standard Therapies

Palmoplantar pustulosis (PPP) is a chronic skin condition affecting the palms of the hands and the soles of the feet. While there is no cure, there are several treatment options to help manage symptoms.

1. First options for treatment:
   • Topical Therapies: These include creams and ointments that are applied directly to the affected areas. Common options are:
     • Topical Steroids: Applied directly to blisters to reduce inflammation
     • Vitamin D Derivatives: Help in reducing the severity of lesions
     • Emollients (Moisturizers): Keep the skin soft and reduce cracking
   • Phototherapy: This involves exposing the skin to certain types of light and includes:
     • UVA and UVB Therapies: Help reduce symptoms with minimal side effects
     • Excimer Laser: Effective, especially at higher doses
     • PUVA Therapy: Combines a drug called psoralen with UVA light, very effective especially for the palms
2. Second option for treatment:
   • If topical treatments and phototherapy don’t work well enough, doctors may prescribe retinoids such as Acitretin. These are vitamin A derivatives that can help improve skin. However, symptoms often return once the treatment stops.
3. Treatment with conventional immunosuppressors:
   • Ciclosporin: Fast-acting medication used for severe cases, but symptoms may return quickly after stopping the treatment
   • Methotrexate: Another option for severe cases, helps by suppressing the immune system
4. Advanced treatments:
   • Biologics: These are newer medications that target specific parts of the immune system. Examples include:
     • TNF-α Inhibitors: Such as infliximab, etanercept and adalimumab
     • IL-17 and IL-23 Blockers: Such as ustekinumab and guselkumab
     • Apremilast: Another effective option
   • Biologics are generally recommended if other treatments don’t work or aren’t suitable for the affected person. They usually need to be taken for over a year, as they work better the longer they are taken, though they may take longer to show results.
5. Emerging treatments:
   • Newer treatments, such as JAK inhibitors, are showing promising results in early reports. However, more research and clinical trials are needed to confirm their effectiveness compared to established treatments.

Additional tips for managing PPP may include:

• Quitting Smoking: Since PPP is common in smokers, quitting can help improve symptoms.
• Avoiding Irritants: Protecting the hands and feet by avoiding contact with irritants like wet work, aggressive soaps and detergents as well as using gloves can help.
• Reducing Stress: Limiting stress may also help improve the symptoms.

In summary, treatment starts with topical therapies and phototherapy. If the initial treatments don’t work well enough, retinoids can be prescribed. Ciclosporin or methotrexate may be used if retinoids cause side effects or don’t work.  Biologics may be used if other options are not suitable, but they tend to work better over longer periods. JAK inhibitors are a potential future option, pending more research.

By working closely with a healthcare provider, the patient and family can find the most suitable treatment plan for managing PPP. It may take some time to see the best results, and sometimes trying different treatments is necessary to find what works best.

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Clinical Trials and Studies

Information on current clinical trials is posted on the Internet at https://clinicaltrials.gov/. All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Toll-free: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]

Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/

For information about clinical trials sponsored by private sources, contact:
https://www.centerwatch.com/

For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/

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References

JOURNAL ARTICLES
Heidemeyer K, May Lee M, Cazzaniga S, Yawalkar N, Naldi L. Palmoplantar pustulosis: a systematic review of risk factors and therapies. Psoriasis (Auckl). 2023 Sep 22;13:33-58. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10522454/

Ramcharran D, Strober B, Gordon K, DeKlotz C, Fakharzadeh S, Yang YW, Swerdel J, Hardin J, Dronavalli S, Paller AS. The Epidemiology of palmoplantar pustulosis: an analysis of multiple health insurance claims and electronic health records databases. Adv Ther. 2023 Nov;40(11):5090-5101. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10567878/

Putra-Szczepaniak M, Maj J, Jankowska-Konsur A, Czarnecka A, Hyncewicz-Gwóźdź A. Palmoplantar pustulosis: Factors causing and influencing the course of the disease. Adv Clin Exp Med. 2020 Jan 28;29(1):157-163.

Twelves S, Mostafa A, Dand N, et al. Clinical and genetic differences between pustular psoriasis subtypes. J Allergy Clin Immunol. 2019 March;143(3):1021–1026.
Sevrain M, Richard MA, Barnetche T, et al. Treatment for palmoplantar pustular psoriasis: systematic literature review, evidence-based recommendations and expert opinion. J Eur Acad Dermatol Venereol. 2014 July 1;28 Suppl 5:13–16.

Brunasso AM, 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
Jun;168(6):1243-51.

INTERNET
Oakley A. Palmoplantar pustulosis. Dermnet NZ. Updated September 2014. https://dermnetnz.org/topics/palmoplantar-pustulosis/. Accessed July 29, 2024.

Palmoplantar Pustulosis on Hands and Feet. WebMD. Reviewed June 10, 2024. https://www.webmd.com/skin-problems-and-treatments/psoriasis/palmoplantar-pustulosis#1. Accessed July 29, 2024.

Palmoplantar pustulosis: Treatment. UptoDate. Reviewed December 2024. https://www.uptodate.com/contents/palmoplantar-pustulosis-treatment. Accessed July 29, 2024.

Palmoplantar pustulosis: Epidemiology, clinical features, and diagnosis. UptoDate. Reviewed June 2024. https://www.uptodate.com/contents/palmoplantar-pustulosis-epidemiology-clinical-features-and-diagnosis?topicRef=93852&source=see_link. Accessed July 29, 2024.

Pustulosis palmaris et plantaris. National Institutes of Health. Genetic and Rare Diseases Information Center. Updated November 2, 2016. https://rarediseases.info.nih.gov/diseases/12820/pustulosis-palmaris-et-plantaris. Accessed July 29, 2024.

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