Rare Immunology News

Disease Profile

Eosinophilic pustular folliculitis

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

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ICD-10

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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.

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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)

Ofuji's disease; Ofuji disease; Eosinophilic folliculitis, pustular;

Categories

Skin Diseases

Summary

Eosinophilic pustular folliculitis (EPF) is a skin disorder characterized by recurring itchy, red or skin-colored bumps and pustules (bumps containing pus).[1] The condition is named after the fact that skin biopsies of this disorder find eosinophils (a type of immune cell) around hair follicles. The papules mostly appear on the face, scalp, neck and trunk and may persist for weeks or months.[1][2] EPF affects males more than females.[3][1]

There are several variants of EPF including classic eosinophilic pustular folliculitis (mainly occurring in adults in Japan); HIV-associated EPF, also referred to as immunosuppression-associated EPF; and infantile EPF (with onset from birth or within the first year of life).[1][3][4] Whether these are distinct disorders rather than variants of one disorder is controversial, partly because the underlying cause of EFP is not known.[1][3]

Several treatments have been described with variable results, including various oral or topical medications and phototherapy. In patients with HIV-associated disease, antiretroviral therapy tends to greatly diminish symptoms or even eliminate the condition.[1][3]

Treatment

A variety of treatment options for eosinophilic pustular folliculitis (EPF) have been described with variable results.[3] Individualized therapy should be tailored to the type of EPF present.[5]

The effectiveness of oral nonsteroidal anti-inflammatory drugs (NSAIDs) is well established for many cases of classic EPF (more than 70%), but the reason why remains unknown. There are cases in which NSAIDs are not effective.[6] For cases of classic EPF, common NSAID options for treatment include indomethacin (orally or topically) and acemetacin. Naproxen has also been used with some success. For classic cases that are not responsive to NSAIDs, other treatment options include:[3][5]

Additional treatment options that may improve symptoms include:[3][1][5]

The infantile form tends to respond well to topical corticosteroid therapy. It is important to recognize that this form typically resolves on it's own before age 3 in over 80% of cases, so observation or avoiding aggressive treatment is often recommended.[5]

In people with HIV-associated EPF, antiretroviral therapy tends to greatly diminish symptoms or even eliminate the condition.[3]

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 Providing General Support

    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

    • 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

      • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
      • 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.
      • PubMed is a searchable database of medical literature and lists journal articles that discuss Eosinophilic pustular folliculitis. Click on the link to view a sample search on this topic.

        References

        1. Scott Barker and Amanda Oakley. Eosinophilic folliculitis. DermNet NZ. April, 2014; https://www.dermnetnz.org/topics/eosinophilic-folliculitis/.
        2. Peter F Weller and Amy D Klion. Eosinophil biology and causes of eosinophilia. UpToDate. Waltham, MA: UpToDate; November, 2016;
        3. Robert A Schwartz. Eosinophilic Pustular Folliculitis. Medscape Reference. June 9, 2016; https://emedicine.medscape.com/article/1070326-overview.
        4. Fujiyama T, Tokura Y. Clinical and histopathological differential diagnosis of eosinophilic pustular folliculitis. J Dermatol. June, 2013; 40(6):419-423.
        5. Nervi SJ, Schwartz RA, Dmochowski M. Eosinophilic pustular folliculitis: a 40 year retrospect. J Am Acad Dermatol. August, 2006; 55(2):285-289.
        6. Katoh M, Nomura T, Miyachi Y, Kabashima K. Eosinophilic pustular folliculitis: a review of the Japanese published works. J Dermatol. January, 2013; 40(1):15-20.
        7. Hai Long, Guiying Zhang, Ling Wang, Qianjin Lu. Eosinophilic Skin Diseases: A Comprehensive Review. Clinical Reviews in Allergy & Immunology. April, 2016; 50(2):189-213.

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