eMedicine Specialties > Dermatology > Diseases of the Adnexa

Eosinophilic Pustular Folliculitis

Author: Marian Dmochowski, MD, Assistant Professor, Department of Dermatology, University School of Medicine at Poznan, Poland
Coauthor(s): Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Contributor Information and Disclosures

Updated: Jan 14, 2009

Introduction

Background

In 1965, Ise and Ofuji described a 42-year-old Japanese housewife with a possible follicular variant of subcorneal pustular dermatosis.1 The patient had crops of follicular pustules on her back, face, chest, and upper arms representing histologic subcorneal pustulosis of the upper hair follicles. The patient also had a leukocytosis of 14,100 white cells/µL, 8% of which were eosinophils. In 1970, Ofuji et al described 3 additional patients and proposed that this new entity be called eosinophilic pustular folliculitis (EPF).2 The use of the term folliculitis has been challenged for this disorder because hair follicles are not seen on the palms or the soles, which may be affected.

Three variants of this disorder have been described: classic eosinophilic pustular folliculitis (as originally described by Ofuji), HIV-associated eosinophilic pustular folliculitis, and infantile eosinophilic pustular folliculitis. Some investigators prefer to consider these 3 distinct disorders. Of the 3, the infantile variety is by far the least characterized. Because the exact nature of these conditions is unknown, whether these conditions are 3 interrelated forms of a single disease or 3 distinct dermatoses is unclear. These conditions share a common pathologic feature, namely a noninfectious eosinophilic infiltration of the hair follicles.

Pathophysiology

A histologic resemblance exists between eosinophilic pustular folliculitis and fungal folliculitis. Some investigators have speculated that eosinophilic pustular folliculitis is due to hyperreactivity to dermatophytes or saprophytic fungi, such as Pityrosporum ovale, in association with a disordered immune system. This concept is supported by the favorable therapeutic response of some patients to oral itraconazole therapy.

The follicle mite, Demodex, has also been considered a possible triggering agent. In certain patients, a combination of Pityrosporum species and Demodex species might play a role in the pathogenesis of the disease. An aberrant helper T-cell type 2 immune response to a follicular antigen, such as Demodex, might be involved in the pathogenesis of HIV-associated eosinophilic pustular folliculitis (see Media File 3). Eosinophilic pustular folliculitis has been described in atopic children with hypersensitivity to Dermatophagoides pteronyssinus.3

An anaerobic organism similar in morphology to Leptotrichia buccalis has been found in one biopsy specimen of a patient with HIV-associated Ofuji disease; the disease responded to oral metronidazole. Others believe that at least the HIV-associated form is an autoimmune disorder with the sebaceous gland cell or a constituent of sebum serving as an autoantigen.

A single case has been reported of a patient with Ofuji disease with pemphiguslike antibody detected by direct immunofluorescence on both lesional skin and healthy skin and by indirect immunofluorescence on human skin but not on guinea pig esophagus.4 Yet another patient with Ofuji disease and high titers of circulating immunoglobulin G and immunoglobulin M antibodies to the cytoplasm of the basal cells of the epidermis and the outer sheath of hair follicles has also been described.5

Another theory is that eosinophilic chemotactic factors from skin surface lipids may be involved.6 A selective migration of leukocyte factor antigen-1–positive eosinophils and lymphocytes to hair follicles may be explained by intercellular adhesion molecule-1 expression by keratinocytes on follicular epithelium but not on epidermis. The expression of endothelial-leukocyte adhesion molecule-1 and vascular cell adhesion molecule-1 by vascular endothelium around hair follicles may also explain this migration.

Eosinophils infiltrating into the dermis and the follicular epidermis express neuronal nitric oxide synthase.7 Activated eosinophils release major basic protein with subsequent tissue damage. In addition to degranulating eosinophils, degranulating mast cells are present in the skin of most patients with HIV-associated eosinophilic folliculitis, which suggests a role for both of these cell types in the pathogenesis of this disease.

Frequency

International

This is an uncommon disorder, except in the AIDS population. The peak incidence of the classic disease is in the second to fourth decades. The peak incidence is usually in the first year of life for the infantile form; eosinophilic pustular folliculitis may be congenital in infantile cases. It may be seen at any age with HIV disease, with an incidence of almost 10% in one survey. Eosinophilic pustular folliculitis is most frequent in association with a low CD4 count.

Mortality/Morbidity

The main morbidity is chronic persistent pruritus, which, especially in the HIV-related form, can interfere with activities of daily living.

Race

All races are affected. The classic form described by Ofuji mainly occurs in Japanese people in Japan. HIV infection is the most common medical condition associated with eosinophilic pustular folliculitis, at least in whites.

Sex

Eosinophilic pustular folliculitis is more common in men than in women.

  • In the classic form, the ratio of men to women is about 5:1.
  • With HIV disease, an overwhelming male prevalence exists.
  • Even in infants, the disease occurs more often in boys than in girls.

Clinical

History

  • The distribution tends to be a seborrheic one on the head and the trunk. About a fifth of patients have palmar and/or plantar plaques, which may be the first sign appearing weeks or months before other clinical features.
  • Lesions are less commonly pruritic with the classic type than in the other 2 forms.

Physical

Patients with eosinophilic pustular folliculitis in the classic form have chronically recurrent crops of sterile follicular papulopustules with peripheral extension and central clearing.8,9

  • Papulopustules with or without plaques tend to favor the face and the trunk, although the extremities may also be involved. With the classic form, the palms and the soles may also be affected. In children, the scalp, particularly at the vertex, is most frequently involved.
  • Some patients may have features of coexistent Ofuji disease and eosinophilic lymphoid granuloma (Kimura disease).10
  • Individual papulopustules may be larger in the classic form, up to 20-50 mm in diameter, rather than the 1-3 mm in diameter seen in patients with HIV disease and in infants; peripheral extension with central clearing may be much less frequent in these 2 forms than in the classic one, which often has an erythematous base. The latter tends to heal more commonly with postinflammatory hyperpigmentation.
  • No systemic involvement is evident, although a peripheral leukocytosis and eosinophilia may be seen. Atypical features, such as nonfollicular papules and urticarial plaques, are often evident in patients with HIV disease and in infants.

Causes

The cause of eosinophilic pustular folliculitis is unknown. Possible etiologies are discussed in Pathophysiology. Reports have described Asian patients in whom eosinophilic pustular folliculitis seemed to be associated with silicone tissue augmentation11 or autologous peripheral blood stem cell transplantation.12 A middle-aged Japanese woman has been described in whom eosinophilic pustular folliculitis was induced by a combination of allopurinol and timepidium bromide as suggested by the results of an oral provocation test with both drugs.13 Moreover, allopurinol alone seemed to induce generalized eosinophilic pustular folliculitis.14

More on Eosinophilic Pustular Folliculitis

Overview: Eosinophilic Pustular Folliculitis
Differential Diagnoses & Workup: Eosinophilic Pustular Folliculitis
Treatment & Medication: Eosinophilic Pustular Folliculitis
Follow-up: Eosinophilic Pustular Folliculitis
Multimedia: Eosinophilic Pustular Folliculitis
References
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References

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Further Reading

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Keywords

eosinophilic pustular folliculitis, EPF, Ofuji's disease, Ofuji disease, eosinophilic folliculitis, HIV-associated eosinophilic folliculitis, HIV-related eosinophilic folliculitis, infantile/childhood eosinophilic pustular folliculitis, sterile eosinophilic pustulosis, eosinophilic pustular dermatosis, infantile/childhood eosinophilic pustulosis of the scalp

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Contributor Information and Disclosures

Author

Marian Dmochowski, MD, Assistant Professor, Department of Dermatology, University School of Medicine at Poznan, Poland
Marian Dmochowski, MD is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Coauthor(s)

Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Takeji Nishikawa, MD, Emeritus Professor, Department of Dermatology, Keio University School of Medicine; Director, Samoncho Dermatology Clinic; Managing Director, The Waksman Foundation of Japan Inc
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio
Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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