Eosinophilic Pustular Folliculitis 

  • Author: Marian Dmochowski; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Aug 10, 2011
 

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 as depicted below. 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.

Widespread hyperpigmented patches on the back and Widespread hyperpigmented patches on the back and the arms in a patient with eosinophilic pustular folliculitis. Courtesy of Professor T. Nishikawa, Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.

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.

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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 the image below). Eosinophilic pustular folliculitis has been described in atopic children[3] with hypersensitivity to Dermatophagoides pteronyssinus. Some authorities have suggested that patients with eosinophilic pustular folliculitis shoud be screened for the presence of coexisting Th2-mediated disorders.[4]

A 51-year-old Japanese man without a concomitant HA 51-year-old Japanese man without a concomitant HIV infection with pustular eosinophilic folliculitis. Courtesy of Professor Akimichi Morita, Nagoya City University Medical School, Nagoya, Japan.

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.[5] 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.[6]

Another theory is that eosinophilic chemotactic factors from skin surface lipids may be involved.[7] 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.[8] 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.

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Epidemiology

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.
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Contributor Information and Disclosures
Author

Marian Dmochowski  MD, PhD, Associate Professor, Department of Dermatology, University School of Medicine at Poznan, Poland

Marian Dmochowski 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, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-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.

Specialty Editor Board

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.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

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.

Jeffrey Meffert, MD  Assistant Clinical Professor of Dermatology, University of Texas School of Medicine at 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.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M 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, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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A patchy lesion on the cheek in a patient with eosinophilic pustular folliculitis. Courtesy of Professor T. Nishikawa, Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.
Widespread hyperpigmented patches on the back and the arms in a patient with eosinophilic pustular folliculitis. Courtesy of Professor T. Nishikawa, Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.
A 51-year-old Japanese man without a concomitant HIV infection with pustular eosinophilic folliculitis. Courtesy of Professor Akimichi Morita, Nagoya City University Medical School, Nagoya, Japan.
Transverse section from a 21-year-old man with eosinophilic pustular folliculitis. A perifollicular inflammatory infiltrate containing numerous eosinophils is present (hematoxylin and eosin, original magnification X400).
Vertical section from a 3-year-old girl with eosinophilic pustular folliculitis. A perifollicular inflammatory infiltrate containing eosinophils is present (hematoxylin and eosin, original magnification X200).
 
 
 
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