Upregulation of IL-36 cytokines in folliculitis and eosinophilic pustular folliculitis.

BACKGROUND: Members of the interleukin (IL)-36 family, IL-36α, IL-36ß and IL-36γ, are potent chemoattractive cytokines for neutrophils and eosinophils. IL-36 receptor antagonist (IL-36Ra) inhibits IL-36α, IL-36ß and IL-36γ activity. However, the immunohistological expression of IL-36α, IL-36ß, 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-36ß, 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-36ß, IL-36γ and IL-36Ra was augmented in both folliculitis and EPF compared with that in normal follicles. Negative correlations were detected between IL-36ß 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-36ß/γ and IL-36Ra was shown in EPF. CONCLUSIONS: The overexpression of IL-36ß, 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.

Facial pustules due to drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms may histopathologically mimic eosinophilic pustular folliculitis: A case report.

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.

Eosinophilic pustular folliculitis of infancy suppressed with cetirizine.

Eosinophilic pustular folliculitis of infancy is a rare inflammatory disease characterized by recurrent, itchy sterile pustules mainly located on the scalp. Topical agents are mostly effective to suppress the symptoms in the majority of cases. However, systemic agents that are safe for long-term use are required for patients with widespread or resistant lesions. We present a case with eosinophilic pustular folliculitis of infancy whose disease was suppressed with cetirizine but recurred after ceasing the drug.

Pilosebaceous targeting by isotretenoin-loaded invasomal gel for the treatment of eosinophilic pustular folliculitis: optimization, efficacy and cellular analysis.

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 32 factorial 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.

A Case of Eosinophilic Pustular Folliculitis Misdiagnosed as Eczema.

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.

Two cases of eosinophilic pustular folliculitis successfully treated with abrocitinib.

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.

A case of generalized eosinophilic pustular folliculitis: treatment with JAK inhibitor.

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.

Two cases of infancy associated eosinophilic pustular folliculitis (I-EPF) comparing the profile of infiltrating cells with classic EPF by immunohistochemical study.

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.

Clinical and Pathological Analysis of 10 Cases of Eosinophilic Pustular Folliculitis.

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.

A Case of Isotopic Response Presented with Eosinophilic Pustular Folliculitis.

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.

Infantile Eosinophilic Pustular Folliculitis in a Child Aged 7 Years: A Case Report.

Eosinophilic pustular folliculitis (EPF) is a rare, chronic, itchy, aseptic disease. Although most cases of infantile EPF (I-EPF) are detected in infants, we found that a 7-year-old child with I-EPF, who received treatment with oral azithromycin in combination with topical narrow bound Ultra Violet B light (NB-UVB) irradiation, with no recurrence at follow-up. Our experience with the successful treatment of this patient can provide a reference for more pediatric patients.

The Role of Reflectance Confocal Microscopy in the Diagnosis and Therapeutic Evaluation of Rare Disease Eosinophilic Pustular Folliculitis.

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.

Classic Eosinophilic Pustular Folliculitis in an Immunocompetent Patient with Syphilis: Are They Related?

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.

A Case of Eosinophilic Pustular Folliculitis since Birth.

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.

A Case of Ofuji Disease Successfully Treated with the Combination of Low-Dose Indomethacin and Topical Tacrolimus.

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: A published work-based comprehensive analysis of therapeutic responsiveness.

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.

Ofuji's disease in an immunocompetent patient successfully treated with dapsone.

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.

Eosinophilic pustular folliculitis: A proposal of diagnostic and therapeutic algorithms.

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.