Eosinophilic pustular folliculitis is a dermatological condition, that involves the formation of pustules in the hair follicles, due to an infiltration of the latter by eosinophils, without the participation of bacteria. It is known to occur and persist in immunosuppressed patients.
Presentation
Eosinophilic pustular folliculitis leads to the development of pustules and papules, caused by an eosinophilic infiltration of hair follicles. Erythema often accompanies the papules and pustules, which mainly appear on the face, in the vast majority of affected patients, and also on the back, upper extremities in the extensor region, neck, and shoulders. Scarring may also occur.
The condition has been strongly associated with certain patient groups, in whom it tends to appear in a more acute way and persist longer. These groups are mainly HIV affected patients and cancer patients, especially those suffering from a hematological malignancy [1] [2]. It also tends to develop in the post-chemotherapy period [3]. Abrasions due to scratching, very extensive erythema and plaques, alongside edematous and erythematous regions (urticaria) are often observed in HIV patients. Individuals affected by eosinophilic pustular folliculitis report an intense itch but constitutional symptoms have not been associated with the condition. Irritability and mild fatigue are the sole non-dermatologic symptoms that have been documented.
The papules and pustules are relatively small, approximately 3mm in diameter and tend to be more localized than generalized. The palms or mucosa do not constitute frequent sites of pustular development [4]. The lesions are expected to self-heal and re-emerge after a period of time, as this is the typical clinical course of the disorder. Ultimately, the papulopustular lesions converge, forming extensive plaques whose center is observed as a healing spot, whereas the peripheral circle expands; lesions may continue to develop anew in these plaques [5].
Eosinophilic pustular folliculitis can also occur in infancy. In this case, the region where papules and pustules are typically expected to develop, is the scalp, in contradistinction to the torso or upper extremities.
Entire Body System
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Constitutional Symptom
Individuals affected by eosinophilic pustular folliculitis report an intense itch but constitutional symptoms have not been associated with the condition. [symptoma.com]
Eyes
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Blurred Vision
vision including corneal deposit and retinal disorder, inflammatory disorder of digestive tract including gastrointestinal perforation and ulcer, cardiac dysrhythmia, chest pain and myocardial infarction, congestive heart failure, cerebrovascular accident [dermaamin.com]
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Corneal Deposit
deposit and retinal disorder, inflammatory disorder of digestive tract including gastrointestinal perforation and ulcer, cardiac dysrhythmia, chest pain and myocardial infarction, congestive heart failure, cerebrovascular accident, epilepsy, hearing [dermaamin.com]
Skin
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Skin Disease
Eosinophilic pustular folliculitis (Ofuji's disease) is a rare skin disease of unknown etiology characterized by infiltrated circinate plaques with sterile follicular pustules in primarily seborrheic areas. [ncbi.nlm.nih.gov]
EPF was recognized as an HIV-related skin disease. [dermquest.com]
Immunodeficiency appears to lead to increased risk of allergic-type skin diseases. [dermnetnz.org]
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Pruritus
This 35-year-old homosexual Japanese man presented with disseminated, discrete, follicular, erythematous papules with intense pruritus over his face, neck, chest wall, and upper back. [ncbi.nlm.nih.gov]
Because pruritus was the prominent symptom rather than pain and tenderness, infectious condition was less suspected. Suspecting EPF, we started her on naproxen, 250 mg twice a day; and after three months, her symptoms greatly improved ( Fig. 1B ). [synapse.koreamed.org]
Sedating forms may be more effective (especially for nocturnal pruritus). Cyproheptadine (Periactin) For the symptomatic relief of allergic symptoms caused by histamine released in response to allergens and skin manifestations. [dermaamin.com]
Pruritus List of cutaneous conditions Erythroderma [en.wikipedia.org]
Uremic pruritus: skin divalent ion content and response to ultraviolet phototherapy. Am J Kidney Dis 1985; 5 (5): 237–41 PubMed Google Scholar 46. Buchness MR, Lim HW, Hatcher VA, et al. [link.springer.com]
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Skin Rash
A 15-year-old, unmarried female presented to our dermatology department for an intensely pruritic skin rash that had appeared abruptly 3 days earlier. [ncbi.nlm.nih.gov]
Eosinophilic folliculitis is a skin disease, primarily characterized by skin rash, itchiness and frequent recurrences. Its exact cause has yet to be identified. [mddk.com]
For many years, this was a rare skin rash, predominately found on the head and neck of Asian patients. However, with the arrival of AIDS, patients began developing lesions which histologically and clinically resembled this rare disease. [thedoctorsdoctor.com]
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Eczema
scabies, eczema) Exposure to poorly chlorinated swimming pools/hot tubs or water contaminated with P. aeruginosa, A. hydrophila, or schistosomes Occlusive corticosteroid use (for multiple hours) Bacteria Most frequently due to S. aureus (increasing [unboundmedicine.com]
It is effective against both staphylococci and streptococci and may be used in the treatment of folliculitis, infected eczema, and as prophylaxis against nasal carriage of staphylococci. [mussenhealth.us]
He had history of atopic eczema in childhood. He gave a history of drug reaction to ibuprofen and diclofenac. He had been treated by a general practitioner with oral antihistamines and topical steroids with no improvement. [idoj.in]
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Pustular Rash
Synonym(s): Ofuji disease. eosinophilic pustular folliculitis, EPF An itchy papular, plaquelike, or pustular rash of uncertain cause, often occurring on the face. [medical-dictionary.thefreedictionary.com]
Face, Head & Neck
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Facial Swelling
CASE REPORT A 60-year-old Japanese woman presented with a 4-month history of facial swelling. [medicaljournals.se]
Psychiatrical
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Suggestibility
For clarification of terminology, we suggest that "eosinophilic folliculitis" is better defined as a pattern histopathologic than as a distinctive disease entity. [ncbi.nlm.nih.gov]
Urogenital
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Bladder Neck Obstruction
neck obstruction; pyloroduodenal obstruction; elderly and/or debilitated patients Pregnancy B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals Precautions May diminish mental alertness; has an atropinelike [dermaamin.com]
Workup
The workup involved in a case suspected of eosinophilic pustular folliculitis includes a complete blood count (CBC), immunoelectrophoresis and a histological analysis of a sample harvested from a lesion, preferably a non-excoriated one.
Affected patients that also suffer from an HIV infection tend to exhibit marked leukopenia, with CD4 levels being less than 250 cells/mL [6]. CBC results will also illustrate eosinophilia, elevated IgE levels and diminished IgG3 and IgA levels [7].
Cultures carried out on the lesion sample that is harvested are expected to reveal no pathogens, since the follicular infiltration is de novo sterile. With reference to the histological characteristics of eosinophilic pustular folliculitis, the following are expected:
- Spongiosis of the follicular epithelium
- Eosinophilic, neutrophilic, lymphocytic and mononuclear infiltration of the outer sheath of the follicle; sebaceous lysis is also observed [3] [8]
- Minor percentage of ruptured follicles
- Follicular mucinosis (possibly)
- Basophilic infiltration in cutaneous regions [9]
- If lesions are present in the soles or hands, subcorneal or intraepidermal pustules are seen
Treatment
If these treatments do not work phototherapy with UVB is the 'gold standard' of treatment and is often curative. [ncbi.nlm.nih.gov]
Prognosis
Including ours, the reported cases of EPF with HIV-negative hematological disease have shown clinical features similar to those of HIV-associated EPF, however the prognosis is better than that for HIV-associated EPF, which usually shows a persistent course [ncbi.nlm.nih.gov]
Prognosis - Eosinophilic pustular folliculitis Not supplied. [checkorphan.org]
(Outcomes/Resolutions) The prognosis of Eosinophilic Folliculitis depends on the extent and severity of the signs and symptoms. Nevertheless, in general, the prognosis is usually good. [dovemed.com]
A male predominance was observed in those with extrafacial eosinophilic pustular folliculitis, with the immunosuppression-associated type more common among them. [22] Prognosis The main morbidity is chronic persistent pruritus, which, especially in the [emedicine.medscape.com]
Etiology
Eosinophilic cellulitis is characterized clinically by an acute dermatitis resembling cellulitis with unknown etiology. [ncbi.nlm.nih.gov]
Epidemiology
BACKGROUND: No epidemiological study has examined the clinical characteristics, including medication use, of patients with eosinophilic pustular folliculitis (EPF). [ncbi.nlm.nih.gov]
Sudden onset of disseminated pustules on trunk Terminology Also called Ofuji disease, eosinophilic folliculitis Epidemiology Typically recurrent disease and spontaneous resolution within months to several years Associated with HIV, lymphoma (non-Hodgkin [pathologyoutlines.com]
Definition and Epidemiology EPF was first reported in 1965 by Ofuji as a follicular variant of subcorneal pustular dermatosis; in 1970, it was termed EPF and was deemed to be a novel clinical entity. [dermquest.com]
Gunter Burg, Werner Kempf Taylor & Francis, 26.05.2005 - 592 Seiten 0 Rezensionen Based on extensive clinical, histological, and experimental studies, this guide serves as an authoritative source of information on the epidemiology, etiology, pathogenesis [books.google.com]
[…] review of Japanese literature. ( 26362415 ) Takamura S....Teraki Y. 2015 18 Eosinophilic pustular folliculitis: The transition in sex differences and interracial characteristics between 1965 and 2013. ( 25675987 ) Nomura T....Miyachi Y. 2015 19 Clinical epidemiology [malacards.org]
Pathophysiology
Abstract Nitric oxide (NO) is produced by a wide variety of human cells and affects physiological and pathophysiological processes. [ncbi.nlm.nih.gov]
Possible etiologies are discussed in Pathophysiology. [dermaamin.com]
Pathophysiology As for the mechanism of eosinophilic accumulation in pilosebaceous units, it has been reported that the eosinophilic chemotactic factor was found in skin-surface lipid products. 7 This appears to be a reasonable explanation because most [dermquest.com]
Prevention
We wish to emphasize the importance of recognizing this self-limiting condition in order to prevent inappropriate antimicrobial treatment. [ncbi.nlm.nih.gov]
It will also provide an easy-to-read guide for the general reader interested in finding out more about the diagnosis and prevention of HIV and AIDS. [books.google.com]
Prevention - Eosinophilic pustular folliculitis Not supplied. Diagnosis - Eosinophilic pustular folliculitis Eosinophilic folliculitis may be suspected clinically when an individual with HIV exhibits the classic symptoms. [checkorphan.org]
Transplantation Substance Use and Addiction Surgery Surgical Innovation Surgical Pearls Teachable Moment Technology and Finance The Rational Clinical Examination Tobacco and e-Cigarettes Toxicology Trauma and Injury Treatment Adherence United States Preventive [jamanetwork.com]
Currently, there are no specific methods or guidelines to prevent Eosinophilic Folliculitis. [dovemed.com]
References
- Suresh MS, Arora S, Nair RR. Doctor I am on fire: eosinophilic folliculitis in HIV negative. Indian J Dermatol Venereol Leprol. 2009 Mar-Apr. 75(2):194-6.
- Fraser SJ, Benton EC, Roddie PH, Krajewski AS, Goodlad JR. Eosinophilic folliculitis: an important differential diagnosis after allogeneic bone-marrow transplant. Clin Exp Dermatol. 2009 Apr; 34(3):369-71.
- Bhandare PC, Ghodge RR, Bhobe MR, Shukla PR. Eosinophilic Pustular Folliculitis Post Chemotherapy in a Patient of Non-Hogkins Lymphoma: A Case Report. Indian J Dermatol. 2015 Sep-Oct; 60(5): 521.
- Tsuboi H, Niiyama S, Katsuoka K. Eosinophilic pustular folliculitis (Ofuji disease) manifested as pustules on the palms and soles. Cutis. 2004 Aug; 74(2):107-10.
- Scavo S, Magro G, Caltabiano R. Erythematous and edematous eruption of the face. Eosinophilic pustular folliculitis. Int J Dermatol. 2010 Sep; 49(9):975-7.
- McCalmont TH, Altemus D, Maurer T, et al. Eosinophilic folliculitis. The histologic spectrum. Am J Dermatopathol. 1995;17:439–46.
- Simpson-Dent S, Fearfield LA, Staughton RC. HIV-associated eosinophilic folliculitis--differential diagnosis and management. Sex Transm Infect. 1999 Oct; 75(5): 291–293.
- McCalmont TH, Altemus D, Maurer T, et al. Eosinophilic folliculitis. The histologic spectrum. Am J Dermatopathol. 1995;17:439–46.
- Satoh T, Ito Y, Miyagishi C, Yokozeki H. Basophils Infiltrate Skin Lesions of Eosinophilic Pustular Folliculitis (Ofuji's Disease). Acta Derm Venereol. 2011 May;91(3):371-2