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
- Sepsis
He had sepsis with lung and liver involvement. EPF is a self-limited dermatosis. On occasion, skin lesions may become superinfected, resulting in localized pyoderma or rarely systemic infection (sepsis). [ncbi.nlm.nih.gov]
Azón-Masoliver A, Casadó J, Brunet J, Martinez MA, del Castillo D: Ofuji’s papuloerythroderma following choledocholithiasis with secondary sepsis: complete resolution with surgery. Clin Exp Dermatol 1998;23:84–86. [karger.com]
- Anemia
aplastic anemia, asthma, bronchospasm, blurred vision including corneal deposit and retinal disorder, inflammatory disorder of digestive tract including gastrointestinal perforation and ulcer, cardiac dysrhythmia, chest pain and myocardial infarction [dermaamin.com]
Skin
- Skin Disease
Indomethacin is a cyclo-oxygenase inhibitor, and shows therapeutic potential for various eosinophilic skin diseases, particularly eosinophilic pustular folliculitis. [ncbi.nlm.nih.gov]
EPF was recognized as an HIV-related skin disease. [dermquest.com]
- 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]
Pruritus List of cutaneous conditions Erythroderma [en.wikipedia.org]
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]
Patients suffering from eosinophilic folliculitis tend to develop sterile papules and pustules recurrently. 50% of the patients develop pruritus, probably with medium intensity. [health.onehowto.com]
Can occur anywhere on the body where hair is found Most frequent symptom is pruritus. [unboundmedicine.com]
- 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]
- Eczema
Commonly Associated Conditions Impetigo Scabies Acne Follicular psoriasis Eczema Xerosis Staphylococcus /MRSA colonization -- To view the remaining sections of this topic, please sign in or purchase a subscription -- Citation Stephens, Mark B., et al. [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]
- 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]
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
Microbiology
- Staphylococcus Aureus
In patient 1 we isolated methicillin-resistant Staphylococcus aureus in a blood culture. He had sepsis with lung and liver involvement. EPF is a self-limited dermatosis. [ncbi.nlm.nih.gov]
Painless or tender pustules, vesicles, or pink/red papulopustules up to 5 mm in size Most commonly infectious in etiology: Staphylococcus aureus bacteria Pseudomonas aeruginosa infects areas of the body exposed to poorly chlorinated hot tubs, pools, or [unboundmedicine.com]
Superficial Staphylococcus aureus folliculitis is more common in children, whilst folliculitis of the beard area is more common in adult males [ 1 ]. Aetiology It can be caused by: Infection: S. aureus is the most common. [patient.info]
Bacterial Infection Infection by the Staphylococcus Aureus bacteria or overgrowth of the Gram-negative bacteria can be a major reason behind Folliculitis. [primehealthchannel.com]
Treatment
Thus, indomethacin should be considered as a first choice of treatment for EPF. [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]
(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]
Prognosis - Eosinophilic pustular folliculitis Not supplied. [checkorphan.org]
Prognosis Nearly all cases will resolve or respond to therapy without sequelae. Cases affecting the immunocompromised and those that are deep or recurrent are more likely to lead to complications. Complications Recurrence. Scarring. [patient.info]
Etiology
Eosinophilic cellulitis is characterized clinically by an acute dermatitis resembling cellulitis with unknown etiology. [ncbi.nlm.nih.gov]
The lack of knowledge about the etiology and pathogenesis of the disease is reflected by the absence of effective treatments. [moh-it.pure.elsevier.com]
Epidemiology
BACKGROUND: No epidemiological study has examined the clinical characteristics, including medication use, of patients with eosinophilic pustular folliculitis (EPF). [ncbi.nlm.nih.gov]
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]
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]
Epidemiology Eosinophilic folliculitis associated with HIV infection typically affects individuals with advanced HIV and low T helper cell counts. [2] It affects both men and women as well as children with HIV and is found throughout the world. [wikidoc.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]
EF may also affect individuals with hematologic disease such as leukemia and lymphoma. [3] It may also affect otherwise normal infants in a self-limited form. [4] Finally, normal individuals can also develop EF — this is more common in Japan. [5] Pathophysiology [wikidoc.org]
Possible etiologies are discussed in Pathophysiology. [emedicine.medscape.com]
Pathophysiology [ edit ] The cause of EF is unknown. [en.wikipedia.org]
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]
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