Rare Endocrinology News
Disease Profile
Eosinophilic pustular folliculitis
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.
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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.
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.
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.
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.
X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.
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.
Not applicable
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
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
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]
- dapsone
- topical and systemic steroids
- isotretinoin
- itraconazole
- permethrin
interferon antibiotics
Additional treatment options that may improve symptoms include:[3][1][5]
- narrowband UVB phototherapy
- ultraviolet therapy with ultraviolet B or with ultraviolet A
- Psoralen plus ultraviolet A (PUVA)
- calcineurin inhibitors such as tacrolimus ointment
- colchicine
- transdermal nicotine patches
The infantile form tends to respond well to topical
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
-
American Academy of Dermatology
1445 New York Ave, NW
Suite 800
Washington, DC 20005
Toll-free: 888-462-DERM (3376)
Fax: 847-240-1859
E-mail: https://www.aad.org/Forms/ContactUs/Default.aspx
Website: https://www.aad.org/
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
- Scott Barker and Amanda Oakley. Eosinophilic folliculitis. DermNet NZ. April, 2014; https://www.dermnetnz.org/topics/eosinophilic-folliculitis/.
- Peter F Weller and Amy D Klion. Eosinophil biology and causes of eosinophilia. UpToDate. Waltham, MA: UpToDate; November, 2016;
- Robert A Schwartz. Eosinophilic Pustular Folliculitis. Medscape Reference. June 9, 2016; https://emedicine.medscape.com/article/1070326-overview.
- Fujiyama T, Tokura Y. Clinical and histopathological differential diagnosis of eosinophilic pustular folliculitis. J Dermatol. June, 2013; 40(6):419-423.
- Nervi SJ, Schwartz RA, Dmochowski M. Eosinophilic pustular folliculitis: a 40 year retrospect. J Am Acad Dermatol. August, 2006; 55(2):285-289.
- 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.
- 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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