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AbstractAim This case study aims to report the efficacy and safety of a Janus kinase (JAK) inhibitor in the treatment of generalized eosinophilic pustular folliculitis (EPF).Methods We present a case of a 16-year-old Chinese patient who had been suffering from EPF for two years and had shown no response to both topical and systemic glucocorticoids. The patient was subsequently treated with oral tofacitinib at a dosage of 5mg daily.Results Significant remission of eruption and pruritus was observed in the patient upon treatment with tofacitinib. However, a relapse occurred upon dose reduction. Subsequent switch to the highly selective JAK1 inhibitor upadacitinib resulted in complete recovery, with the patient achieving a symptom-free status after six months.Conclusions JAK inhibitors show promise as a potential treatment option for EPF patients who do not respond to traditional therapies.

Yuan Li,1,2 Xianxu Yang,3 Jiejie Lu,1 Kai Chen,3 Weiwei Wu,3 Ming Zhang,3 Wanni Li1 1Department of Cosmetic Dermatology, The Fifth People’s Hospital of Hainan Province, Haikou, Hainan, People’s Republic of China; 2Department of Dermatology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, People’s Republic of China; 3Department of Dermatology, The Fifth People’s Hospital of Hainan Province, Haikou, Hainan, People’s Republic of ChinaCorrespondence: Wanni Li, Department of Cosmetic Dermatology, The Fifth People’s Hospital of Hainan Province, No. 8 Longhua Road, Longhua District, Haikou, 570100, Hainan, People’s Republic of China, Email liwanni1204@outlook.comAbstract: Eosinophilic pustular folliculitis (EPF) is a rare skin disease. The typical skin lesions of classic EPF are localized on the upper limbs’ face, trunk, and extensor surfaces. However, when the skin lesions initially appear on the palms and soles, it is not easy to diagnose as EPF. Here, we report a case of a male patient who presented with erythematous plaques, pustules, and crusted erosions on the hands and feet, which were misdiagnosed as eczema ten years ago. Over ten years, the patient experienced recurrent erythematous patches with bumps and itching on the face, trunk, and extremities after improving the hand and foot lesions. A histopathological biopsy was performed to establish a definitive diagnosis, revealing pustules formed by eosinophilic infiltrates within the hair follicles. The diagnosis was confirmed as eosinophilic pustular folliculitis. The patient was treated with oral indomethacin at a dosage of 75mg/day, resulting in the disappearance of the skin lesions. After a 3-month follow-up, no recurrence was observed.Keywords: eosinophilic pustular folliculitis, indomethacin

Infancy associated eosinophilic pustular folliculitis (I‐EPF) is a clinical variant of EPF that develops in childhood. Previous studies have suggested that I‐EPF exhibits clinical and histological differences distinct from other variants, including classic EPF. Herein, we report two patients with I‐EPF treated with topical indomethacin. These two cases exhibited less perifollicular and more perivascular eosinophilic infiltration, which is different in distribution from that of classic EPF. Immunohistochemical study demonstrated that the infiltrating mononuclear cells were CD4‐dominant T cells in classic EPF and I‐EPF, whereas the number of CD68‐positive cells was significantly higher in classic EPF than in I‐EPF. Immunohistochemical staining was also performed for eosinophilic pustular folliculitis (HPGDS), which has been reported to induce eosinophils and is a therapeutic target of indomethacin in classic EPF. HPGDS‐positive cells were also observed in I‐EPF, which may explain the effectiveness of topical indomethacin. Although clinical and histopathological features of I‐EPF are different from other variants, the arachidonic acid pathway could be involved in eosinophil infiltration, not only in classic EPF but also in I‐EPF. [ABSTRACT FROM AUTHOR]

Yuan Li,1,2,* Gaihe Chen,3,* Xin Zhou,4,* Xiaole Zheng,1,* Ming Zhang,3 Xiaojuan Yao,1 Jiejie Lu,1 Xiaohuan Hu1 1Department of Cosmetic Dermatology, The Fifth People’s Hospital of Hainan Province, Haikou, Hainan, People’s Republic of China; 2Department of Dermatology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, People’s Republic of China; 3Department of Dermatology, The Fifth People’s Hospital of Hainan Province, Haikou, Hainan, People’s Republic of China; 4Medical Administration Department, The Fifth People’s Hospital of Hainan Province, Haikou, Hainan, People’s Republic of China*These authors contributed equally to this workCorrespondence: Xiaohuan Hu; Jiejie Lu, Department of Cosmetic Dermatology, The Fifth People’s Hospital of Hainan Province, No. 8 Longhua Road, Longhua District, Haikou, Hainan, 570100, People’s Republic of China, Email 872114382@qq.com; lujiejie677@163.comAbstract: We conducted a retrospective analysis of clinical and pathologic data from January 2020 to June 2023, focusing on 10 patients diagnosed with eosinophilic pustular folliculitis at our dermatology clinic. Four of the ten patients had the first rash on the face, five on the trunk, and one on the palms and feet, all of which were initially scattered papules that gradually increased and fused into erythematous plaques with a circular distribution. Seven had pustules with small surface desquamation, and three cases had micro swelling on the face. The rash involved only the face in 5 cases, the face and trunk in 5 cases, and the face, trunk, hands, and feet in 1 case. Seven of the ten patients were pruritic, and 3 had no obvious pruritus. The histopathological features were mild epidermal hyperplasia, lymphocytic and eosinophilic infiltration around the superficial middle dermal vessels and appendages, and eosinophilic and neutrophilic abscesses in the local hair follicles. Treatment with oral indomethacin, prednisone, and minocycline was effective.Keywords: eosinophilic pustular folliculitis, eosinophils, indomethacin

Yuan Li,1,2,* Miaoting Wu,1,* Xiaohuan Hu,1,* Gaihe Chen,1,* Xiaojuan Yao,1 Xin Zhou,3 Jiejie Lu1 1Department of Cosmetic Dermatology, The Fifth People’s Hospital of Hainan Province, Haikou, Hainan, People’s Republic of China; 2Department of Dermatology, The First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu, People’s Republic of China; 3Medical Administration Department, The Fifth People’s Hospital of Hainan Province, Haikou, Hainan, People’s Republic of China*These authors contributed equally to this workCorrespondence: Jiejie Lu, Department of Cosmetic Dermatology, The Fifth People’s Hospital of Hainan Province, No. 8 Longhua Road, Longhua District, Haikou, Hainan, 570100, People’s Republic of China, Email lujiejie677@163.com Xin Zhou, Medical Administration Department, The Fifth People’s Hospital of Hainan Province, No. 8 Longhua Road, Longhua District, Haikou, Hainan, 570100, People’s Republic of China, Email zx5575759@126.comAbstract: Eosinophilic pustular folliculitis (EPF) is a rare skin disease for which the gold standard of diagnosis relies on the invasive examination of pathological tissue sections. However, due to its invasive nature, many patients tend to refuse this diagnostic test. In such situations, reflectance confocal microscopy (RCM) can be a valuable diagnosis tool. Reflectance confocal microscopy (RCM) can accurately identify the specific structures for biopsy and provide objective imaging data to evaluate clinical symptoms following treatment. Therefore, we present a case report demonstrating the utility of RCM in diagnosing and assessing the treatment of the rare disease EPF for reference.Keywords: eosinophilic pustular folliculitis, minocycline, reflectance confocal microscopy, non-invasive detection

Lulu Xv,* Bin Wang,* Qing Zhu, Guoqiang Zhang* The First Hospital of Hebei Medical University, Shijiazhuang, China*These authors contributed equally to this workCorrespondence: Guoqiang Zhang, No. 89, DongGang Road, Shijiazhuang, Hebei Province, People’s Republic of China, Tel +8618633888122, Email zlx090702@163.comAbstract: This is a 58-year-old woman who has had itching on her right back for 10 days; a month ago she developed blisters on her right back with no apparent cause and pain, and was diagnosed with “herpes zoster”. Erythema and blisters with itching reappeared 10 days ago on the right side of the back, where the original blisters had subsided. The histopathological manifestations of the lesions were: mild hyperkeratosis of the epidermis, pustules within the stratum corneum, irregular proliferation of the spinous layer, spongiosis, local destruction of the basal layer, infiltration of eosinophils, neutrophils, histiocytes and scattered plasma cells in the dermis and subcutis. According to the medical history, clinical manifestations and histopathology of the lesions, the diagnosis was Wolf’s isotopic response after herpes zoster.Keywords: Wolf’s isotopic response, herpes zoster, eosinophilic pustular folliculitis

Yuchen Li,* Ruxiao Nie,* Xianwei Cao, Chuan Wan Department of Dermatology, The First Affiliated Hospital of Nanchang University, Nanchang, People’s Republic of China*These authors contributed equally to this workCorrespondence: Chuan Wan, Department of Dermatology, The First Affiliated Hospital of Nanchang University, Yong Wai Zheng Street 17#, Nanchang, 330006, People’s Republic of China, Tel +86-18070052970, Email chuanwan@ncu.edu.cnAbstract: Eosinophilic pustular folliculitis (EPF) is a rare, chronic, non-infectious inflammatory skin disease. Although the pathogenesis of EPF is unknown, eosinophilic pustular folliculitis may be associated with human immunodeficiency virus (HIV) infection, malignancies or syphilis. Here, we report the first case of EPF associated with syphilis, indicating that syphilis and EPF are correlated with T-helper type 2 immune responses. A 48-year-old man gradually developed erythema and pustules on the face, neck. Physical examination revealed multiple infiltrative red patches and plaques on the face, neck with tiny pustules. Skin biopsy results revealed that the dermal follicular sebaceous gland unit was infiltrated by a large number of neutrophils and eosinophils, forming eosinophilic microabscesses. Therefore, the patient was diagnosed with EPF associated with syphilis and received drug treatment. After the treatment, the pustules markedly decreased, leaving behind pigmentation. Furthermore, the patient is still being followed up.Keywords: eosinophilic pustular folliculitis, secondary syphilis, pathogenesis, T-lymphocyte immune response

Introduction: Eosinophilic pustular folliculitis (EPF), sometimes called Ofuji disease, is a chronic skin disorder of unknown etiology which develops predominantly in elderly man. Clinically it usually presents as itching centrifugally extending papulopustular rash. Aim: To present a case of EPF and to raise dermatologists' awareness of this this dermatosis when dealing with patients who are in higher risk of EPF occurrence. Case study: A 90-year-old man presented to the dermatology clinic due to the itching rash that had been present for 3 months. Physical examination of the patient revealed red-brown papulopustules covering trunk and extremities, most severe on the frontal part of thighs. Other possible causative agents of the symptoms were excluded. Histopathological results together with clinical symptoms led us to the final diagnosis of classic EPF Results and discussion: EPF is challenging to diagnose. The disease is very rare, especially in Europe, and clinical and/or histological appearance may mimic other dermatological conditions. The most commonly affected areas are face, back and trunk. EPF is usually classified into three main subtypes: classic, infantile and immunosuppression-associated. There are many viable therapeutic algorithms for the treatment of EPF. Conclusions: Eosinophilic pustular folliculitis should be considered in differential diagnosis of pruritic follicular papulopustular rash affecting elderly men. Histopathological results play a crucial role in the making of final diagnosis. [ABSTRACT FROM AUTHOR]

Abstract is missing (Short communication)

Competing Interests: None disclosed.

An elderly woman developed pruritic follicularpapules over a period of 5 years. A biopsy revealedfocal intraepidermal eosinophilic pustules, leading tothe diagnosis of Ofuji disease or eosinophilic pustularfolliculitis. A discussion of this condition is presented.

Eosinophilic pustular folliculitis (EPF) is classified into Ofuji disease, immunosuppression-associated EPF, and infancy-associated EPF. The association of EPF with Demodex infestation is rarely reported. We report five cases of EPF with Demodex overgrowth. All patients were young females presenting with recurrent, itchy papulopustules on the face for 2 months to 2 years. Laboratory tests revealed eosinophilia (n = 2) and iron deficiency anemia (n = 1). Skin biopsy of papulopustular lesions was performed in each patient, and all revealed folliculocentric infiltration with eosinophils. Infundibular pustules containing Demodex mites were found in two patients. All patients had high Demodex densities examined by superficial needle-scraping method (7–18 mites/5 pustules) and/or thumbnail-squeezing method (37–255 mites/cm2). Based on the clinical and laboratory findings, the patients were either first treated as having demodicosis or as having EPF. According to the treatment responses, the cases might represent Demodex-induced EPF or EPF-like reaction (Cases 1–3) or demodicosis superimposed on EPF (Cases 4 and 5). In sum, we report five cases of EPF with Demodex overgrowth (demodicosis). These cases illustrate that the possibility of Demodex overgrowth should be considered in cases of EPF with incomplete treatment response to indomethacin and/or oral corticosteroids. Conversely, EPF should be considered in cases of demodicosis with incomplete responses to antiparasitic treatment.

Aim: This case study aims to report the efficacy and safety of a Janus kinase (JAK) inhibitor in the treatment of generalized eosinophilic pustular folliculitis (EPF)., Methods: We present a case of a 16-year-old Chinese patient who had been suffering from EPF for two years and had shown no response to both topical and systemic glucocorticoids. The patient was subsequently treated with oral tofacitinib at a dosage of 5mg daily., Results: Significant remission of eruption and pruritus was observed in the patient upon treatment with tofacitinib. However, a relapse occurred upon dose reduction. Subsequent switch to the highly selective JAK1 inhibitor upadacitinib resulted in complete recovery, with the patient achieving a symptom-free status after six months., Conclusions: JAK inhibitors show promise as a potential treatment option for EPF patients who do not respond to traditional therapies.

Eosinophilic pustular folliculitis (EPF) (Ofuji disease) is a chronic, noninfectious pruritic cutaneous disorder of unknown etiology. No official guidelines are available for its treatment. Herein we present the case of a 59-year-old Caucasian man admitted to our outpatient clinic due to a generalized itchy skin rash characterized by papulo-pustules involving the face, trunk, and limbs. Histological examination supported the clinical diagnosis of EPF (Ofuji disease). The combination of low-dose oral indomethacin and topical tacrolimus ointment once a day led to a complete resolution of the lesions as well as associated symptoms in 8 weeks.

Eosinophilic pustular folliculitis (EPF) is classified into Ofuji disease, immunosuppression-associated EPF, and infancy-associated EPF. The association of EPF with Demodex infestation is rarely reported. We report five cases of EPF with Demodex overgrowth. All patients were young females presenting with recurrent, itchy papulopustules on the face for 2 months to 2 years. Laboratory tests revealed eosinophilia (n = 2) and iron deficiency anemia (n = 1). Skin biopsy of papulopustular lesions was performed in each patient, and all revealed folliculocentric infiltration with eosinophils. Infundibular pustules containing Demodex mites were found in two patients. All patients had high Demodex densities examined by superficial needle-scraping method (7–18 mites/5 pustules) and/or thumbnail-squeezing method (37–255 mites/cm2). Based on the clinical and laboratory findings, the patients were either first treated as having demodicosis or as having EPF. According to the treatment responses, the cases might represent Demodex-induced EPF or EPF-like reaction (Cases 1–3) or demodicosis superimposed on EPF (Cases 4 and 5). In sum, we report five cases of EPF with Demodex overgrowth (demodicosis). These cases illustrate that the possibility of Demodex overgrowth should be considered in cases of EPF with incomplete treatment response to indomethacin and/or oral corticosteroids. Conversely, EPF should be considered in cases of demodicosis with incomplete responses to antiparasitic treatment. [ABSTRACT FROM AUTHOR]

A newborn male infant presented with multiple pustules and erosions with erythema involving his scalp and forehead at birth. One week after birth, new pustules continued to appear, forming crusted, ring-shaped plaques with pigmentation. Tests for possible pathogens were negative. Tzanck smear and skin biopsy revealed pustules beneath the stratum corneum at sites corresponding to hair follicles, which contained eosinophils and neutrophils. Taken together, a diagnosis of eosinophilic pustular folliculitis (EPF) was made. The pustules on the head disappeared rapidly with topical corticosteroid treatment, although new eruptions were still observed on the trunk about one month after birth. To our knowledge, only two cases of EPF since birth have been reported to date. Here, we also discuss the differential diagnosis of noninfectious pustular diseases at birth, including erythema toxicum neonatorum and transient neonatal pustular melanosis. These diseases, and EPF, may present with very similar clinical symptoms at birth, and the Tzanck test or biopsy may be required for differential diagnosis. [ABSTRACT FROM AUTHOR]

Eosinophilic pustular folliculitis (EPF) is a rare, non-infectious, inflammatory disease characterized by an eosinophil-dominated infiltrate within and around pilosebaceous units. Sometimes, EPF manifests with eruptions in follicle-free areas, although it is not common, and treatment may be difficult. In this case study we report two patients with refractory EPF who presented with eruptions of both classic follicle areas and follicle-free areas. These two patients were successfully treated with abrocitinib after treatment failure with several traditional therapies, such as indomethacin, steroids, and cyclosporin. One patient achieved complete remission at week 4 and the other at week 1, with no reported adverse effects. Therefore, we believe that abrocitinib may be a viable and safe therapeutic option for refractory EPF., (© 2024 Japanese Dermatological Association.)

Keywords: eosinophilic pustular folliculitis; food allergy; infancy EN eosinophilic pustular folliculitis food allergy infancy 1 3 3 01/05/22 20220101 NES 220101 To the Editor, Eosinophilic pustular folliculitis (EPF) of infancy (EPFI) is an inflammatory dermatosis that mostly occurs in early infancy and resolves spontaneously by 3 years of age.1 Prominent eosinophilic infiltration, itchy papulopustules measuring 1-3 mm accompanied by erythema, recurrent outbreaks, and usually involvement of scalp are the characteristic findings of the disease.1,2 The best way to quickly diagnose EPF without the assessment of the clinical course is to perform a Tzanck smear of a pustule or skin biopsy.3 The pustules in EPF contain a large number of eosinophils and are accompanied by dermal infiltration of eosinophil-dominated inflammatory cells around the hair follicles.4 Eosinophilia is common, and serum IgE levels may also be raised.1 EPFI is the least well-described type of EPF, and neither the etiology nor the pathogenesis is clarified yet.5 The disease is associated with a variety of different factors,1,2 but there is no case report that shows an association with food allergy. From the history of the patient, it was learned that when he was 23 days old, he was hospitalized in the intensive care unit for 1 week with the diagnosis of pneumonia and received the treatment of ampicillin and gentamicin. Depending on the severity of egg allergy, most patients develop tolerance to egg within 2-5 years,10 so it is planned to try stopping the diet slowly after the patient reaches 24 months of age. [Extracted from the article]

Background: Members of the interleukin (IL)‐36 family, IL‐36α, IL‐36β and IL‐36γ, are potent chemoattractive cytokines for neutrophils and eosinophils. IL‐36 receptor antagonist (IL‐36Ra) inhibits IL‐36α, IL‐36β and IL‐36γ activity. However, the immunohistological expression of IL‐36α, IL‐36β, IL‐36γ and IL‐36Ra has never been addressed in normal follicles, folliculitis or eosinophilic pustular folliculitis (EPF). Methods: We performed immunohistochemical staining for IL‐36α, IL‐36β, IL‐36γ and IL‐36Ra using 10 cases of EPF, nine of non‐specific folliculitis, 10 normal skin samples and 10 samples of normal follicles adjacent to a sebaceous naevus as a control. Two dermatologists, who were blind to the patient records, evaluated all of the slides. Results: The immunoreactive IL‐36α was hardly detected in the follicular epithelium and epidermis in the normal skin, folliculitis or EPF. The expression of IL‐36β, IL‐36γ and IL‐36Ra was augmented in both folliculitis and EPF compared with that in normal follicles. Negative correlations were detected between IL‐36β and IL‐36Ra and between IL‐36γ and IL‐36Ra in normal follicles; however, these were absent in folliculitis. In contrast to normal follicles and folliculitis, a significant positive correlation between IL‐36β/γ and IL‐36Ra was shown in EPF. Conclusions: The overexpression of IL‐36β, IL‐36γ and IL‐36Ra is an integral part of the inflammatory response of folliculitis and EPF. The coordinated expression of IL‐36γ and IL‐36Ra may be related to the pathomechanism of EPF. [ABSTRACT FROM AUTHOR]

Pustules with facial and/or neck edema is one characteristic feature of drug‐induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms (DIHS/DRESS) at the early stage. Although several retrospective histopathologic studies on DIHS/DRESS have been reported, the detailed histopathologic findings of facial pustules for DIHS/DRESS are unavailable. We herein report a case of DIHS/DRESS with facial pustules that was histopathologically similar to eosinophilic pustular folliculitis (EPF). Eosinophilic infiltration into expanded follicles and sebaceous glands, which is highly characteristic of EPF, was detected in pustules due to DIHS/DRESS in this case. There are numerous pathophysiological similarities between DIHS/DRESS and EPF, which may cause their histopathologic similarity. Our findings suggest that facial pustules of DIHS/DRESS may histopathologically mimic EPF. [ABSTRACT FROM AUTHOR]

Eosinophilic pustular folliculitis or Ofuji's disease is a non-infectious eosinophilic infiltration of hair follicles, which usually presents with itchy papules and pustules in a circinate configuration. We report this case of an immunocompetent patient with erythematous papules and plaques without macropustules diagnosed as eosinophilic pustular folliculitis—a rarely reported entity outside Japan. He was successfully treated with oral dapsone.

Context:Eosinophilic pustular folliculitis is a secondary symptom associated with HIV infection appears as levels of CD4 lymphocyte cells and T4 lymphocyte cell. Isotretinoin, an analog of vitamin A (retinoid) alters the DNA transcription mechanism and interferes in the process of DNA formation. It also inhibits the eosinophilic chemotactic factors present in sebaceous lipids and in the stratum corneum of patients suffering from this ailment. Objective:The present research was aimed to formulate isotretenoin-loaded invasomal gel to deliver and target the drug to pilosebaceous follicular unit. Methods:Nine invasomal formulations (F1–F9) were prepared applying 32factorial designs and characterized. Results:Formulation F9 was selected as optimized formulation due to optimum results and highest %CDP of 85.94 ± 1.86% in 8 h. Transmission electron microscopy (TEM) suggested uniformity in vesicles shape and size in F9 and developed as invasomal gel (IG). Limitations:Clinical phase-I, phase-II, and phase-III studies will be required before using on human patients. Conclusion:Confocal laser scanning microscopy (CLSM) validates that IG successfully reaches the pilosebaceous follicular unit and further studied on cell line (SZ-95) exhibited IC50 of ≤8 (25 μM of isotretenoin). Cell cycle analysis confirmed IG arrested the cell growth up to 82% with insignificant difference to pure isotretenion. [ABSTRACT FROM PUBLISHER]

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Eosinophilic pustular folliculitis (EPF) is a chronic intractable pruritic dermatosis. Although indomethacin is generally effective against EPF and considered as a first-line therapy, quite a few patients with indomethacin still suffer from the symptoms. Among other therapeutic options, some antibiotics have been reported to be effective; however, there has been no epidemiological description regarding oral antibiotics use in patients with EPF. In this study, we investigated the frequency of antibiotics use and the effectiveness in patients with EPF.

Eosinophilic pustular folliculitis(EPF),also known as Ofuji's disease, is a distinctive dermatosis characterized by the appearance of follicular papules or pustules involving the face, trunk and/or extremities. Its variants include a classic type, an HIV-associated type, an infantile type. We here report on a patient with classic EPF, who was successfully treated with oral indomethacin. One year follow up of this case revealed dramatic improvement

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 immunosuppression-associated EPF ( IS- EPF), which may be referred to dermatologists in inpatient services for assessments. Infancy-associated 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Eosinophilic pustular folliculitis (EPF) occurs in patients with hematological disorders. However, clinical information about hematological disorder-associated EPF is scarce. We report two cases of EPF associated with mantle cell lymphoma and reviewed the available published work on Japanese cases. We identified a total of 23 Japanese cases, including the two cases reported here, who had hematological disorder-associated EPF. Fourteen cases were associated with treatment for hematological malignancies (transplantation-related EPF) and nine cases were associated with hematological malignancies themselves (hematological malignancy-related EPF). Although the skin eruption was clinically indistinguishable between the two subtypes, transplantation-related EPF occurred on the face and trunk of young and middle-aged men and women, whereas hematological malignancy-related EPF occurred mostly on the face of older men. Peripheral blood eosinophilia was more frequently observed in transplantation-related EPF. These observations suggest variations among patients with EPF associated with hematological disorders. [ABSTRACT FROM AUTHOR]

Eosinophilic skin diseases, commonly termed as eosinophilic dermatoses, refer to a broad spectrum of skin diseases characterized by eosinophil infiltration and/or degranulation in skin lesions, with or without blood eosinophilia. The majority of eosinophilic dermatoses lie in the allergy-related group, including allergic drug eruption, urticaria, allergic contact dermatitis, atopic dermatitis, and eczema. Parasitic infestations, arthropod bites, and autoimmune blistering skin diseases such as bullous pemphigoid, are also common. Besides these, there are several rare types of eosinophilic dermatoses with unknown origin, in which eosinophil infiltration is a central component and affects specific tissue layers or adnexal structures of the skin, such as the dermis, subcutaneous fat, fascia, follicles, and cutaneous vessels. Some typical examples are eosinophilic cellulitis, granuloma faciale, eosinophilic pustular folliculitis, recurrent cutaneous eosinophilic vasculitis, and eosinophilic fasciitis. Although tissue eosinophilia is a common feature shared by these disorders, their clinical and pathological properties differ dramatically. Among these rare entities, eosinophilic pustular folliculitis may be associated with human immunodeficiency virus (HIV) infection or malignancies, and some other diseases, like eosinophilic fasciitis and eosinophilic cellulitis, may be associated with an underlying hematological disorder, while others are considered idiopathic. However, for most of these rare eosinophilic dermatoses, the causes and the pathogenic mechanisms remain largely unknown, and systemic, high-quality clinical investigations are needed for advances in better strategies for clinical diagnosis and treatment. Here, we present a comprehensive review on the etiology, pathogenesis, clinical features, and management of these rare entities, with an emphasis on recent advances and current consensus. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]