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Infectious Diseases > MEDICAL TOPICS
Eosinophilic Folliculitis
Article Last Updated: Feb 28, 2006
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
Author: M Angelica Selim, MD, Associate Director of Dermatopathology, Departments of Pathology and Internal Medicine, Assistant Professor, Duke University Medical Center
Coauthor(s):
Christopher R Shea, MD, Professor and Chief, Section of Dermatology, Department of Medicine, University of Chicago
Editors: Joseph Richard Masci, MD, Chief of Infectious Diseases, Associate Director, Associate Professor, Department of Internal Medicine, Division of Infectious Diseases, Elmhurst Hospital Center, Mount Sinai School of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; Joseph F John Jr, MD, FACP, FIDSA, FSHEA, Professor of Medicine, Molecular Genetics and Microbiology, Medical University of South Carolina; Associate Chief of Staff for Education, Ralph H Johnson Veteran's Administration Medical Center; Eleftherios Mylonakis, MD, Clinical and Research Fellow, Department of Internal Medicine, Division of Infectious Diseases, Massachusetts General Hospital; Michael E Zevitz, MD, Assistant Professor of Medicine, Finch University of the Health Sciences, The Chicago Medical School; Consulting Staff, Private Practice
Author and Editor Disclosure
Synonyms and related keywords:
eosinophilic folliculitis, EF, eosinophilic pustular dermatosis, eosinophilic pustular folliculitis, Ofuji disease, Ofuji's disease, sterile eosinophilic pustulosis
Background
Eosinophilic folliculitis (EF) is a recurrent skin disorder of unknown etiology. In 1965, Ise and Ofuji reported a case of recurrent follicular pustules and eosinophilia in a Japanese woman. Five years later, and after 3 additional cases, Ofuji named this skin condition eosinophilic pustular folliculitis. Orfanos and Sterry argued that the name "sterile eosinophilic pustulosis" might be more appropriate because this lesion is not restricted to the hair follicle. Other names have also been proposed (eg, classic form of EF, Ofuji disease, eosinophilic pustular dermatosis). Over the past 2 decades, the spectrum of EF has expanded to pediatric populations, transplant recipients, and persons with HIV and hematopoietic disorders.
Pathophysiology
Although the exact etiology of EF remains obscure, studies have favored an autoimmune process directed against sebocytes or some component of sebum. Markers of acute inflammatory activation of the epithelia, such as ICAM-1 and MAC 387, are strongly positive in sebocytes of EF lesions but only weakly reactive in the follicular epithelium. Antibody formation and the creation of immune complexes are believed to directly or indirectly mediate clinical manifestations. Patients with EF make antibodies to the intercellular substance of the lower epidermis and the outer root sheath of the hair follicle. An abnormal Th2-type immune response to a follicular antigen, such as caused by Demodex species, may be responsible for HIV-associated EF.
Frequency
United States
The prevalence of EF is unknown.
Mortality/Morbidity
EF is not disabling or life threatening, but it may be intensely pruritic.
Race
The prevalence of EF is higher among Asians, but it also occurs in persons of Hispanic descent and in whites and blacks.
Sex
- Men have a 5-fold increased frequency of EF.
- HIV-associated EF is more common among homosexual or bisexual men.
Age
- Disease onset usually occurs in the third-to-fifth decades of life.
- In the pediatric population, EF typically affects patients aged 5-10 months although neonatal cases have been reported.
History
- Patients with EF develop recurrent crops of sterile pustules and papules.
- Pruritus is present in half of patients.
- The clinical course is characterized by multiple cycles of exacerbations and remissions.
- The individual skin lesions usually heal spontaneously in a few months to several years.
- This skin disorder can become severe and persistent in certain populations (eg, persons infected with HIV).
Physical
- EF typically appears as an area of erythematous papules and pustules and involves the face in 85% of patients.
- Other locations include the back and the extensor surface of the upper extremities.
- The papules gradually become confluent, creating indurate polycyclic plaques with a healing center and spreading periphery. They ultimately fade away, leaving residual hyperpigmentation and scaling.
- Atypical presentations or nonclassic forms occur in certain populations.
- Infantile EF is characterized by erythematous papulopustules on the scalp as the primary area of involvement.
- Patients with HIV-associated EF present with widespread urticarial lesions or large erythematous plaques with excoriations.
- Involvement of mucosa and the palms and soles has been rarely reported.
Causes
- Although production of cytokines and chemotactic factors and expression of intercellular adhesion molecules are evidence of activation of the follicular sebaceous unit, the stimuli that provoke these changes are unknown.
- Many observations suggest a role for immunologic, infectious, and environmental factors.
- Brenner et al described 3 cases of EF associated with Pseudomonas infection of the hair follicles; the lesions improved with antipseudomonal treatment and recurred upon cessation of therapy.
- Other investigators have reported infectious associations including dermatophyte, larva migrans, Pityrosporum, and retrovirus.
- In addition, EF has been associated with a variety of medical conditions, including lymphoma, leukemia, myelodysplastic syndrome, atopy, polycythemia vera, and bone marrow transplantation.
Candidiasis
Urticaria
Other Problems to be Considered
- Classic form of EF
- Acneiform eruptions
- Follicular eczema
- Folliculitis (bacterial, fungal, or dermatophytic)
- Pustular psoriasis
- Subcorneal pustular dermatosis (Sneddon-Wilkinson)
- Alopecia mucinosis
- Pediatric EF
- Acropustulosis of infancy
- Scabies
- Scalp pyoderma
- Neonatal EF
- Erythema toxicum neonatorum
- Miliaria
- Neonatal acne
- Neonatal varicella
- Transient neonatal pustular melanosis
- HIV-associated EF
- Pruritic papular eruption of HIV
- Reactions to arthropod bites and stings
Lab Studies
- Complete blood cell count reveals leukocytosis and eosinophilia.
- Immunoelectrophoresis reveals elevated levels of immunoglobulin E (IgE), low levels of immunoglobulin G3 (IgG3), and low levels of immunoglobulin A (IgA) in pediatric EF.
- Culture of skin lesion reveals no consistent bacterial or fungal growth.
- CD4 count in patients with HIV who have EF is usually less than 250-300 cells/mm3.
Histologic Findings
The infundibulum of the hair follicle manifests eosinophilic spongiosis and pustulosis. The infiltrate often extends to the adjacent sebaceous gland. Although most follicles are preserved, some follicular walls are destroyed by the inflammatory infiltrate. The infiltrate is mainly composed of eosinophils with variable numbers of neutrophils and mononuclear cells. It also manifests a moderately dense, perivascular, and perifollicular inflammatory infiltrate comprised of eosinophils, lymphocytes (mainly CD4+), and macrophages. Follicular mucinosis may been seen in lesions of EF. Special stains for microorganisms are negative. The lesions of the palms and soles have subcorneal and intraepidermal pustules, accompanied by a variable dermal inflammatory infiltrate.
Medical Care
- Numerous topical and systemic therapies are available for EF. Treatment modalities are chosen on the basis of disease severity, patient preference (including cost and convenience), and response.
- Topical corticosteroids are the mainstay of treatment of EF.
- The mechanism of action of corticosteroids in this condition is not fully understood; the anti-inflammatory and immunosuppressive properties of these agents may contribute to their effect.
- The potency of the steroid prescribed depends upon the location of the skin lesions. In the scalp, potent steroids in alcohol solution, such as fluocinonide 0.05%, are frequently indicated. On the face and other sensitive body sites, a low potency cream, such as hydrocortisone 1%, may suffice.
- The typical regimen consists of twice-daily application of topical corticosteroids. Most patients show a decrease in inflammation and plaques. Skin atrophy from topical corticosteroid use is usually not a problem unless the medication is continuously applied after the skin has returned to a normalized state. Severe flares may be treated with short courses of oral prednisone.
- Retinoids, such as isotretinoin, inhibit sebaceous gland function and keratinization. Clinical improvement occurs in association with a reduction in sebum secretion.
- This effect is temporary and is related to the dose and duration of treatment.
- Monitoring for hypertriglyceridemia and hepatotoxicity is required.
- Common adverse effects include cheilitis and alopecia. Systemic retinoid therapy is teratogenic; it is only indicated in men and in women who have no reproductive potential.
- Alternatives include indomethacin and dapsone.
- In patients with HIV, treat mild disease with topical steroids and oral antihistaminics. Treat moderate disease with oral itraconazole, isotretinoin, or phototherapy. Treat severe disease with isotretinoin therapy for several months.
- Potential treatments include oxyphenbutazone, colchicine, minocycline, acitretin, cyclosporine A, UV-B therapy, interferon alfa-2b, tacrolimus, doxycycline, and radiation therapy.
Consultations
- Consider referral to a dermatologist in the following settings:
- If confirmation of the diagnosis is needed
- If the response to treatment is inadequate
- If the primary care physician is not familiar with the recommended treatment modality
- If the patient has widespread or severe disease
The goals of pharmacotherapy are to reduce morbidity and to prevent complications.
Drug Category: Corticosteroids
Have both anti-inflammatory (glucocorticoid) and salt-retaining (mineralocorticoid) properties. Glucocorticoids have profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.
| Drug Name | Prednisone (Deltasone, Orasone, Liquid Pred) |
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| Description | May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. |
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| Adult Dose | 5-60 mg/d PO |
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| Pediatric Dose | 0.05-2 mg/kg/d PO |
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| Contraindications | Documented hypersensitivity; viral infection, peptic ulcer disease, hepatic dysfunction, connective tissue infections, and fungal or tubercular skin infections; GI disease |
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| Interactions | Coadministration with estrogens may decrease clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics |
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| Pregnancy | B - Usually safe but benefits must outweigh the risks.
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| Precautions | Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur with glucocorticoid use |
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Drug Category: Retinoids
Decrease cohesiveness of abnormal hyperproliferative keratinocytes and may reduce potential for malignant degeneration. Modulate keratinocyte differentiation. Reduce risk of skin cancer formation in renal transplant patients.
| Drug Name | Isotretinoin (Accutane) |
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| Description | Retinoid acid derivative reduces size of sebaceous gland and decreases sebum production. Also regulates cell differentiation and proliferation. |
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| Adult Dose | 0.5 mg/kg PO divided bid |
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| Pediatric Dose | Not established |
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| Contraindications | Documented hypersensitivity |
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| Interactions | Toxicity may occur with vitamin A coadministration; pseudotumor cerebri or papilledema may occur when coadministered with tetracyclines; isotretinoin may reduce plasma levels of carbamazepine |
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| Pregnancy | X - Contraindicated in pregnancy
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| Precautions | May decrease night vision; inflammatory bowel disease may occur; may be associated with development of hepatitis; occasional exaggerated healing response of acne lesions (excessive granulation with crusting) may occur; diabetic patients may experience problems in controlling their blood sugar while on isotretinoin; avoid exposure to UV light or sunlight until tolerance achieved; discontinue treatment if rectal bleeding, abdominal pain, or severe diarrhea occur; mood swings or depression may occur; caution if history of depression |
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Drug Category: Nonsteroidal anti-inflammatory agents (NSAIDs)
Have analgesic, anti-inflammatory, and antipyretic activities. Mechanism of action is not known but may inhibit cyclooxygenase activity and prostaglandin synthesis. Other mechanisms may exist as well (eg, inhibition of leukotriene synthesis, lysosomal enzyme release, lipoxygenase activity, neutrophil aggregation, various cell-membrane functions).
| Drug Name | Indomethacin (Indocin, Indochron ER) |
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| Description | Rapidly absorbed; metabolism occurs in liver by demethylation, deacetylation, and glucuronide conjugation; inhibits prostaglandin synthesis. |
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| Adult Dose | 75 mg PO divided bid/tid; not to exceed 200 mg |
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| Pediatric Dose | 1-2 mg/kg/d PO divided bid; not to exceed 4 mg/kg/d |
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| Contraindications | Documented hypersensitivity; GI bleeding or renal insufficiency |
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| Interactions | Coadministration with aspirin increases risk of inducing serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity of NSAIDs; may decrease effect of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; may increase PT when taking anticoagulants (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; phenytoin levels may be increased when administered concurrently |
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| Pregnancy | B - Usually safe but benefits must outweigh the risks.
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| Precautions | Category D in third trimester of pregnancy; acute renal insufficiency, hyperkalemia, hyponatremia, interstitial nephritis, and renal papillary necrosis may occur; increases risk of acute renal failure in patients with preexisting renal disease or compromised renal perfusion; reversible leukopenia may occur, (discontinue with persistent leukopenia, granulocytopenia, or thrombocytopenia)
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Drug Category: Antibiotics, sulfone; Leprostatic agents
May improve the clinical stage of the disease.
| Drug Name | Dapsone (Avlosulfon) |
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| Description | Bactericidal and bacteriostatic against mycobacteria; mechanism of action is similar to that of sulfonamides in which competitive antagonists of PABA prevent formation of folic acid, thus inhibiting bacterial growth. |
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| Adult Dose | 100 mg PO qd |
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| Pediatric Dose | 1-2 mg/kg PO qd; not to exceed 100 mg/d |
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| Contraindications | Documented hypersensitivity; known G-6-PD deficiency |
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| Interactions | May inhibit anti-inflammatory effects of clofazimine; hematologic reactions may increase with folic acid antagonists, eg, pyrimethamine (monitor for agranulocytosis during the second and third months of therapy); probenecid increases toxicity; trimethoprim with dapsone may increase toxicity of both drugs; because of increase in renal clearance, levels may significantly decrease when administered concurrently with rifampin |
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| Pregnancy | C - Safety for use during pregnancy has not been established.
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| Precautions | Perform weekly CBC counts (first mo); then perform WBC counts monthly (6 mo); then semiannually; discontinue if significant reduction in platelets, leukocytes, or hematopoiesis is observed; caution in methemoglobin reductase deficiency, G-6-PD deficiency (patients receiving >200 mg/d), or hemoglobin M due to high risk for hemolysis and Heinz body formation; caution in patients exposed to other agents or conditions (eg, infection, diabetic ketosis) capable of producing hemolysis; peripheral neuropathy can occur (rare); phototoxicity may occur when exposed to UV light |
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Complications
- EF is a cutaneous disease without important sequelae. The only residual expression of the disease is cutaneous hyperpigmentation and scaling.
Prognosis
- EF is a benign dermatologic disease; however, EF is associated with advanced AIDS, and there is an increased risk of developing opportunistic infections in patients who are HIV positive.
Medical/Legal Pitfalls
- Failure to recognize and treat opportunistic infections in patients with EF who are HIV positive
| Media file 1:
Eosinophilic pustular folliculitis in a patient who is HIV positive. Note acneiform hyperpigmented papules. Photograph courtesy of Sarah A. Myers, MD. |
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| Media file 2:
Eosinophilic pustular folliculitis in a patient who is HIV positive (the same patient shown in picture 1). Note follicular-based excoriated papules and pustules on trunk. Photograph courtesy of Sarah A. Myers, MD. |
 | View Full Size Image | |
Media type: Photo
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| Media file 3:
Eosinophilic folliculitis (low power). Note eosinophilic spongiosis, particularly involving the infundibular region of the hair follicle. |
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Media type: Photo
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| Media file 4:
Eosinophilic folliculitis (high power). In addition to the abundant eosinophils, note the variable numbers of neutrophils and mononuclear cells. |
 | View Full Size Image | |
Media type: Photo
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Eosinophilic Folliculitis excerpt Article Last Updated: Feb 28, 2006
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