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

et al. 2016

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Eosinophilic pustular folliculitis (EPF) is a sterile inflammatory dermatosis of unknown etiology. In addition to classic EPF, which affects otherwise healthy individuals, an immunocompromised state can cause immunosuppressionassociated EPF (IS-EPF), which may be referred to dermatologists in inpatient services for assessments. Infancyassociated EPF (I-EPF) is the least characterized subtype, being observed mainly in non-Japanese infants. Diagnosis of EPF is challenging because its lesions mimic those of other common diseases, such as acne and dermatomycosis. Furthermore, there is no consensus regarding the treatment for each subtype of EPF. Here, we created procedure algorithms that facilitate the diagnosis and selection of therapeutic options on the basis of published work available in the public domain. Our diagnostic algorithm comprised a simple flowchart to direct physicians toward proper diagnosis. Recommended regimens were summarized in an easy-to-comprehend therapeutic algorithm for each subtype of EPF. These algorithms would facilitate the diagnostic and therapeutic procedure of EPF.

Section: Introductionmentioning

confidence: 70%

Eosinophilic pustular folliculitis: A proposal of diagnostic and therapeutic algorithms

et al. 2016

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“…This tendency was confirmed in a nationwide survey we conducted in Japan between 2010 and 2011 in which the number of men and women was nearly equal (72 vs 73). 41 The reason why the female ratios have gradually increased is not clear.…”

Section: Chronology Of I-epfmentioning

confidence: 99%

Eosinophilic pustular folliculitis: The transition in sex differences and interracial characteristics between 1965 and 2013

et al. 2015

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Eosinophilic pustular folliculitis (EPF) is characterized by a non-infectious infiltration of eosinophils in the hair follicles. It has three variants: (i) classic EPF; (ii) immunosuppression-associated EPF, which herein is subdivided into HIV-associated (IS/HIV) and non-HIV-associated (IS/non-HIV); and (iii) infancy-associated EPF (I-EPF). The rarity of EPF has hindered our understanding of this entity. To examine the characteristics of EPF, with respect to age, sex, race, and chronology, published in case reports to date, we queried PubMed using the following terms: ("eosinophilic pustular folliculitis" [All Fields] OR "eosinophilic folliculitis" [All Fields]) AND ("1965/1/1" [PDAT]: "2013/12/31" [PDAT]). Additional Japanese cases were collected from Igaku Chuo Zasshi through Ichushi-Web, JDream III, and secondhand quotations from domestic periodicals published in Japan. Proceedings were excluded. The PubMed search produced 275 citations containing 358 cases of EPF (224 men, 132 women, and two of unspecified sex); these cases involved classic EPF (101 Japanese and 81 non-Japanese), IS/HIV (4 Japanese and 85 non-Japanese), IS/non-HIV (4 Japanese and 20 non-Japanese), and I-EPF (4 Japanese and 59 non-Japanese). Ichushi generated an additional 148 citations containing 207 cases of Japanese (148 men and 59 women), which included cases of classic EPF (181 cases), IS/HIV (14 cases), IS/non-HIV (9 cases), and I-EPF (3 cases). There was no sex difference in the classic EPF cases reported between 2003 and 2013, whereas IS/HIV, IS/non-HIV, and I-EPF were predominated by men. There is room for reconsideration of sex differences, particularly with regard to classic EPF. The rarity and specificity of I-EPF in Japan may reflect a state of uncertainty about this entity.

“…Second, there could be a significant population of female EPF sufferers who have not been correctly diagnosed, because there was no sex difference in reported patients with EPF over the past few decades, in contrast to the impression that EPF affects males predominantly . This observation was confirmed by a nationwide survey conducted by us, which showed that women constituted 49.3% of the 145 outpatients with EPF who had been treated between 2010 and 2011 at 67 facilities registered as major teaching hospitals by the Japanese Dermatological Association in Japan . Furthermore, patients with malignancies or hematological disorders, who develop IS/non‐HIV, may be overlooked and thus never receive proper treatment.…”

Section: Discussionmentioning

confidence: 99%

“…4,6 This observation was confirmed by a nationwide survey conducted by us, which showed that women constituted 49.3% of the 145 outpatients with EPF who had been treated between 2010 and 2011 at 67 facilities registered as major teaching hospitals by the Japanese Dermatological Association in Japan. 10 Furthermore, patients with malignancies or hematological disorders, who develop IS/non-HIV, may be overlooked and thus never receive proper treatment. To avoid this possibility, IS/non-HIV must be ruled out when pruritic papules emerge during the course of IRIS after BMT or PBSCT.…”

Section: B) Howmentioning

confidence: 99%

“…In a previous published work‐based analysis, we reported that 78% (35/45) of Japanese patients with classic EPF were successfully treated by oral indomethacin . This tendency was confirmed by a nationwide cross‐sectional study conducted in Japan between 2010 and 2011, in which oral indomethacin was effective in 92.3% of 104 Japanese patients with EPF . However, we have less information on the efficacies of other regimens for EPF.…”

Section: Introductionmentioning

confidence: 99%

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Eosinophilic pustular folliculitis: A published work‐based comprehensive analysis of therapeutic responsiveness

et al. 2016

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Eosinophilic pustular folliculitis (EPF) is a non-infectious inflammatory dermatosis of unknown etiology that principally affects the hair follicles. There are three variants of EPF: (i) classic EPF; (ii) immunosuppression-associated EPF, which is subdivided into HIV-associated (IS/HIV) and non-HIV-associated (IS/non-HIV); and (iii) infancy-associated EPF. Oral indomethacin is efficacious, especially for classic EPF. No comprehensive information on the efficacies of other medical management regimens is currently available. In this study, we surveyed regimens for EPF that were described in articles published between 1965 and 2013. In total, there were 1171 regimens; 874, 137, 45 and 115 of which were applied to classic, IS/HIV, IS/non-HIV and infancy-associated EPF, respectively. Classic EPF was preferentially treated with oral indomethacin with efficacy of 84% whereas topical steroids were preferred for IS/HIV, IS/non-HIV and infancy-associated EPF with efficacy of 47%, 73% and 82%, respectively. Other regimens such as oral Sairei-to (a Chinese-Japanese herbal medicine), diaminodiphenyl sulfone, cyclosporin and topical tacrolimus were effective for indomethacin-resistant cases. Although the preclusion of direct comparison among cases was one limitation, this study provides a dataset that is applicable to the construction of therapeutic algorithms for EPF.