Eosinophilic pustular folliculitis: A review of the Japanese published works

Abstract

Eosinophilic pustular folliculitis (EPF), also known as Ofuji's disease, is an inflammatory dermatosis that was first described in Japan in 1970. More than 300 cases have been reported so far, and 113 Japanese cases have been reported in Japan since 1980. To comprehend the characteristics of Japanese EPF cases, we classified these cases into three types: classic, immunosuppression-associated (IS-EPF), and infancy-associated (I-EPF). Trends in age of onset and in distribution and characterization of eruptions differed between the types. We found 91 cases of classic EPF (mean age, 39.7 years), consisting of 66 males (73%) and 25 females (27%), in most of which eruptions primarily affected the face; 18 cases of IS-EPF (44.2 years), consisting of 15 males (83%) and three females (17%), in which eruptions affected the face less predominantly; and four cases of I-EPF (7.0 years), consisting of two males (50%) and two females (50%), primarily affecting the scalp. The number of IS-EPF cases has increased since the late 1990s, reflecting the increasing number of HIV-positive patients in Japan. Systemic nonsteroidal anti-inflammatory drugs were effective in more than 70% of cases. Dimethyl diphenyl sulfone, antibiotics including minocycline, psoralen plus ultraviolet A therapy and ultraviolet B treatments worked in some cases. Topical steroids and tacrolimus were also effective in some cases of EPF, while topical indomethacin was less effective.

Clinical Epidemiology of Eosinophilic Pustular Folliculitis: Results from a Nationwide Survey in Japan

“…Although several cases of infants with EPF (also known as I-EPF) have been reported, no patient was aged <10 years in the present study [13,14,15]. Katoh et al [3] reviewed all published cases of EPF in Japan and showed that 4% were I-EPF, suggesting that I-EPF may include other diseases than EPF because of the lower incidence and the unusual clinical findings. At least, our results suggest that I-EPF was a relatively rare type of EPF, even if such an entity exists.…”

“…Previous studies reported that EPF was more likely in men [3,12]. However, another study suggested that the approximate male:female ratio is 1:1.6 [8].…”

“…There has been a growing number of case reports of IS-EPF in Japan accompanying the increase in HIV patients, but the prevalence of HIV infection in Japan remains quite low (approximately 0.02%) [16]. A previous review of Japanese reports suggested that 16% of all EPF cases had HIV infection [3]. We predicted that the actual proportion of IS-EPF would be much smaller than 16% after discounting the effect of publication bias.…”

“…EPF typically manifests as clusters of pruritic, follicular and sterile pustules and papules distributed on the face and neck, which is known as classic EPF [3]. In addition, two other types of EPF that are associated with human immunodeficiency virus (HIV) infection and infancy are known as immunosuppression-associated EPF (IS-EPF) and infancy-associated EPF (I-EPF), respectively [4,5].…”

Eosinophilia in Dermatologic Disorders

“…Biopsies show spongiosis of hair follicle epithelium with infiltration of eosinophils and lymphocytes, forming eosinophilic follicular pustules that are a typical diagnostic feature. [54][55][56] In addition to classical EPF, mainly found in Japan, infancy-associated EPF and immunosuppression-associated EPF in the setting of human immunodeficiency virus infection and malignancies have been described. Classical EPF predominantly affects the face and trunk, and infancy-associated EPF the scalp.…”