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Disease Profile

Pustulosis palmaris et plantaris

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

Age of onset

-

ICD-10

L40.3

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

Palmoplantar pustulosis; LPP; PPP;

Categories

Congenital and Genetic Diseases; Skin Diseases

Summary

Pustulosis palmaris et plantaris (PPP) is a rare and chronic inflammatory condition that primarily affects the palms of the hands and soles of the feet. Signs and symptoms of the condition generally develop during adulthood and include crops of pustules on one or both hands and/or feet that erupt repeatedly over time. Affected individuals may also experience itching (pruritus), pain, or a burning sensation.[1][2][3] The classification of PPP is controversial. Some have proposed that PPP is a variant of psoriasis, while others believe it to be a separate disease.[2][4] Though the area of involvement is often limited, PPP can have a significant effect on quality of life and can interfere with walking or other daily activities.[2][3] PPP often has a chronic and relapsing course and may be resistant to treatment.[4] Treatment options may include topical steroids, emollients, certain medications called retinoids, and treatment with light (phototherapy).[2][3][4][5]

Cause

The cause of pustulosis palmaris et plantaris (PPP) is not fully understood, though many theories have been proposed. Some have suggested PPP may be a disorder of the sweat glands.[1][2] Environmental factors are believed to play a part as well. For instance, there is a strong association between smoking and PPP. Other environmental factors that may contribute to PPP include stress, infections (especially tonsillitis and sinusitis), and certain medications. Some have also suggested an association with allergies to certain metals.[2][5] Although most cases occur in people with no family history of the condition, more than one family member can be affected. This suggests that genetic factors may contribute to the development of the condition in some cases. [1][2]

Treatment

Although there is no cure for pustulosis palmaris et plantaris, various measures may be useful to manage symptoms. General suggestions include smoking cessation and emollients (non-cosmetic moisturizers) to soften dry skin and prevent cracking. Topical corticosteroids (steroids), an oral retinoid such as  acitretin, and photochemotherapy (ultraviolet radiation treatment) are often first-line treatments. [1][2] Other methods that may be utilized when initial treatment options fail include a combination of oral retinoid and photochemotherapy or immunosuppressive therapy with medications such as cyclosporine.[2]

Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

    Learn more

    These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

    Where to Start

    • British Association of Dermatologists has information on palmoplantar pustulosis.
    • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.

      In-Depth Information

      • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
      • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
      • PubMed is a searchable database of medical literature and lists journal articles that discuss Pustulosis palmaris et plantaris. Click on the link to view a sample search on this topic.

        References

        1. Oakley A. Palmoplantar pustulosis. DermnetNZ. September, 2014; https://www.dermnetnz.org/scaly/palmoplantar-pustulosis.html.
        2. Giovanna Brunasso, MD; Cesare Massone, MD. Palmoplantar pustulosis: Epidemiology, clinical features, and diagnosis. UpToDate. October, 2016;
        3. Giovanna Brunasso, MD; Cesare Massone, MD. Palmoplantar pustulosis: Treatment. UpToDate. November, 2014; https://www.uptodate.com/contents/palmoplantar-pustulosis-treatment.
        4. Misiak-Galazka, M., Wolska, H, and Rudnicka, L. What do we know about palmoplantar pustulosis?. J Eur Acad Dermatol Venereol.. August 13, 2016; https://www.ncbi.nlm.nih.gov/pubmed/27521275.
        5. Raposo, I and Torres, T.. Palmoplantar Psoriasis and Palmoplantar Pustulosis: Current Treatment and Future Prospects. . Am J Clin Dermatol.. August, 2016; 17(4):349-58. https://www.ncbi.nlm.nih.gov/pubmed/27113059.

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