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Last updated:
April 15, 2020
Years published: 2020
NORD gratefully acknowledges Alexandra M.G. Brunasso Vernetti, MD, Dermatology Unit, Galliera Hospital, Genoa, Italy, for assistance in the preparation of this report.
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 women between 40 and 69 years of age, and predominantly 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 for patients who 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 is recorded in 10-42% of patients affected by 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.
PPP causes recurrent crops of sterile pustules to form on the palms of the hands and/or the soles of the feet The pustules often coalesce in an erythematous background where infiltrated and scaly plaques tends to form after several days with the onset of painful fissures. Itching and/or pain are often present. Patients may also feel like their hands and/or feet are burning. Due to these symptoms, patients may have difficulty walking and performing other daily tasks with the hands and feet.
The exact cause of PPP is not known. Researchers have found some possible causes including smoking, infections, certain medications and genetics.
PPP mostly affects women between 40 and 69 years of age and smokers, but this condition may affect people of any age.
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 to test it for histopathology.
Although there is no definitive cure for PPP but there are different treatment options available.
Possible treatment options include:
For severe cases, immunosuppressants like methotrexate or cyclosporine may be used. If these medications are not tolerated or inefficient, biologicals like TNF-alpha blockers (etanercept, adalimumab mainly), anti IL-12/23 inhibitor (Ustekinumab), anti IL-23 inhibitors (mainly Guselkumab) and anti IL-17 may be prescribed.
Since PPP is common in smokers, quitting smoking may help symptoms improve. Avoidance of contact irritants (avoid wet work, use gloves, avoid using aggressive soaps and detergents) is also recommended. Limiting stress may also help symptoms get better.
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: prpl@cc.nih.gov
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/
JOURNAL ARTICLES
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 March 1, 2020.
Palmoplantar Pustulosis on Hands and Feet. WebMD. Reviewed November 13, 2018. https://www.webmd.com/skin-problems-and-treatments/psoriasis/palmoplantar-pustulosis#1. Accessed March 1, 2020.
Palmoplantar pustulosis: Treatment. UptoDate. Reviewed December 2019. https://www.uptodate.com/contents/palmoplantar-pustulosis-treatment. Accessed March 1, 2020.
Palmoplantar pustulosis: Epidemiology, clinical features, and diagnosis. UptoDate. Reviewed June 2019. https://www.uptodate.com/contents/palmoplantar-pustulosis-epidemiology-clinical-features-and-diagnosis?topicRef=93852&source=see_link. Accessed March 1, 2020.
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 February 23, 2020.
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Learn more https://rarediseases.org/patient-assistance-programs/medicalert-assistance-program/Ensuring that patients and caregivers are armed with the tools they need to live their best lives while managing their rare condition is a vital part of NORD’s mission.
Learn more https://rarediseases.org/patient-assistance-programs/rare-disease-educational-support/This first-of-its-kind assistance program is designed for caregivers of a child or adult diagnosed with a rare disorder.
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