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Eosinophilic Pustular Folliculitis

Eosinophilic Pustulosis, Eosinophilic Pustular Folliculitis Ofuji

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.

Constitutional Symptom
  • Individuals affected by eosinophilic pustular folliculitis report an intense itch but constitutional symptoms have not been associated with the condition.[symptoma.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]
  • 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]
  • Pruritus List of cutaneous conditions Erythroderma[en.wikipedia.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]
  • 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]
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]
Pustular Rash
Facial Swelling
  • CASE REPORT A 60-year-old Japanese woman presented with a 4-month history of facial swelling.[medicaljournals.se]
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]
  • The excellent response to ketoconazole treatment suggests a possible pathogenic role for Pityrosporum.[ncbi.nlm.nih.gov]
  • Although EPF was initially described as an idiopathic disease, the association of some cases with immunologic alterations or diseases, such as immunodeficiencies, suggests a possible immunopathologic event in the pathogenesis of EPF.[ncbi.nlm.nih.gov]
  • We suggest that the immunologic abberations ascribed to myelodysplastic syndrome and the increase in the eosinophil cell line in the bone marrow might play roles in the development of EPF in our case.[ncbi.nlm.nih.gov]
  • However, a fungal cause is suggested by (1) tinea-like annular lesions, (2) initial clinical consideration of fungal folliculitis in many reported cases, (3) an association with the acquired immunodeficiency syndrome, and (4) an association with scaly[ncbi.nlm.nih.gov]

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]
  • Thus, indomethacin should be considered as a first choice of treatment for EPF.[ncbi.nlm.nih.gov]
  • The effectiveness of each treatment was assessed by a severity score index. Eleven patients were treated with oral indomethacin, and the severity scores of all patients were decreased after the treatment.[ncbi.nlm.nih.gov]
  • Our results suggest that transdermal nicotine patches may be useful and safe in the treatment of EPF.[ncbi.nlm.nih.gov]
  • Resolution of the rash took place during the 8 weeks following cessation of allopurinol and treatment with oral and topical corticosteroids.[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]
  • It is important to recognize this form of EPF after HSCT and differentiate it from graft-versus-host disease since it responds readily to topical steroids and appears to have an excellent prognosis. 2017 Wiley Periodicals, Inc.[ncbi.nlm.nih.gov]
  • 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]
  • PROGNOSIS AND TREATMENT CHARACTERIZATION PROGNOSTIC FACTORS TREATMENT GENERAL Ofuji's disease: diagnostic and therapeutic problems. A report of three cases. Patrone P, Bragadin G, Stinco G, De Francesco V, Frattasio A.[thedoctorsdoctor.com]

Etiology
  • Eosinophilic pustular folliculitis (EPF) is a cutaneous inflammatory follicular disorder of unknown etiology. The diagnosis is made on the basis of clinical and histopathologic features.[ncbi.nlm.nih.gov]
  • Eosinophilic cellulitis is characterized clinically by an acute dermatitis resembling cellulitis with unknown etiology.[ncbi.nlm.nih.gov]
  • 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]
  • Laboratory tests disclosed no infectious or parasitic etiology. Histological examination showed eosinophilic pustular folliculitis (EPF) in each of the 3 cases. The lesions responded well to topical corticosteroids.[ncbi.nlm.nih.gov]
  • Eosinophilic pustular folliculitis (EPF) (Ofuji's disease) is a rare dermatosis of 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]
Sex distribution
Age distribution

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]
  • 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]
  • 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]
  • 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]
  • Comedone formation occurs when increased cohesiveness prevents the shedding of keratinocytes, and they accumulate in the opening, leading to hyperkeratosis. Increased production of follicular keratinocytes also occurs.[clinicaladvisor.com]
  • […] with tacrolimus is helpful in suppressing response to immune system Phototherapy with UVB and UVA light is shown to give promising results Corticosteroids, calcineurin inhibitors, colchicines and retinoids are administered to relieve the condition and prevent[health.onehowto.com]

References

Article

  1. 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.
  2. 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.
  3. 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.
  4. 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.
  5. 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.
  6. McCalmont TH, Altemus D, Maurer T, et al. Eosinophilic folliculitis. The histologic spectrum. Am J Dermatopathol. 1995;17:439–46.
  7. Simpson-Dent S, Fearfield LA, Staughton RC. HIV-associated eosinophilic folliculitis--differential diagnosis and management. Sex Transm Infect. 1999 Oct; 75(5): 291–293.
  8. McCalmont TH, Altemus D, Maurer T, et al. Eosinophilic folliculitis. The histologic spectrum. Am J Dermatopathol. 1995;17:439–46.
  9. 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

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Last updated: 2019-07-11 20:02