巨大型嗜酸性脓疱性毛囊炎治愈1例

45岁男性患者，头面部丘疹、脓疱、肿块反复4年，双小腿褐色斑块1月，外周血嗜酸粒细胞计数增高，皮损组织病理显示表皮内毛囊内嗜酸性脓肿，皮质类固醇联合抗生素及抗组胺药治疗疗效显著。     

The ultrastructural histopathology of eosinophilic pustular folliculitis.

The follicular skin lesions of a patient with eosinophilic pustular folliculitis were investigated by electron microscopy. Pustules in the outer root sheath contained acantholytic keratinocytes with numerous microvilli and features of desmosomal cleavage. The infiltrating eosinophils and neutrophils exhibited autolytic or degenerative changes rather than degranulation. The aggregated tubulo-vesicular structures were associated with the debris of autolytic eosinophils. Multiple, tiny, bubble-like structures enclosed within a membrane were frequently seen in the intercellular space. The intercellular space of the outer root sheath was widened with decreased desmosomal adhesion between the keratinocytes, but no intracellular edema was detectable. The infiltrating lymphocytes, predominantly T-cells with convoluted nuclei, extended cytoplasmic processes to adjacent keratinocytes. Apposition of T-lymphocytes and Langerhans cells was noted. Some keratinocytes in the outer root sheath contained large, sebaceous lipid droplets. No obvious virus particles or other pathogenic agents were detected. It is possible that T-lymphocytes and other immunosurveillance cells are involved in the pathomechanism of eosinophilic pustular folliculitis.     

Transdermal nicotine patches for eosinophilic pustular folliculitis

We previously reported the clinical effectiveness of transdermal nicotine patches for the treatment of skin disorders with eosinophilic infiltration such as Kimura's disease, erythema nodosum and eosinophilic pustular folliculitis (EPF). We assessed the efficacy and safety of transdermal nicotine patches for EPF. We treated eight patients with EPF with transdermal nicotine patches and evaluated the treatment response by performing overall lesional assessment. Excellent 77and good responses were obtained in five and one patient(s), respectively. In the other two patients, the lesions remained unchanged. No severe adverse effects were observed. Our results suggest that transdermal nicotine patches may be useful and safe in the treatment of EPF.     

HIV seronegative eosinophilic pustular folliculitis successfully treated with doxicycline

Eosinophilic pustular folliculitis (EPF) is an unusual disease, first described in adult East Asians in 1970 by Ofuji. It is characterized by follicular papules and pustules tending to coalesce and form plaques involving the trunk, face and extremities. In recent years, it has been often associated with human immunodeficiency virus (HIV) infection or with immunosuppressed and/or oncohaematological patients. EPF has been described in immunocompetent adult caucasian patients only occasionally. The diagnosis requires clinical and microbiological features such as sterile folliculitis and histopathological findings characterized by folliculitis and perifolliculitis with eosinophilic infiltrate. We describe an HIV seronegative caucasian male with EPF, allergic to non-steroidal anti-inflammatory drugs and indomethacin, treated with oral doxicycline. The treatment led to the complete remission of the lesions within 2 months.     

嗜酸性脓疱性毛囊炎

嗜酸性脓疱性毛囊炎主要可分为3种类型，即经典型、免疫抑制相关型和婴儿型，许多患者的发病与艾滋病有关。该文就嗜酸性脓疱性毛囊炎的分类、流行病学、发病机制、临床表现、实验室检查、鉴别诊断和治疗等各方面作一较全面的介绍。     

Clinical and histopathological differential diagnosis of eosinophilic pustular folliculitis

Eosinophilic pustular folliculitis (EPF) is an inflammatory disease characterized by repeated pruritic follicular papules and pustules arranged in arcuate plaques, and folliculotropic infiltration of eosinophils. The diagnosis of EPF is occasionally difficult and problematic because EPF may share the clinical appearance and histological findings with other diseases. Moreover, EPF has several clinical subtypes, including the classical type, infantile type and immunosuppression‐associated type. Because the therapies of EPF are relatively specific as compared to eczematous disorders, accurate diagnosis is essential for the management of EPF. Clinical differential diagnoses include tinea, acne, rosacea, eczematous dermatitis, granuloma faciale, autoimmune annular erythema, infestations and pustular dermatosis. Histologically, cutaneous diseases with eosinophilic infiltrates can be differentially diagnosed. Follicular mucinosis, mycosis fungoides and other cutaneous T‐cell lymphomas are the most important differential diagnoses both clinically and histopathologically. It should be kept in mind particularly that the initial lesions of cutaneous T‐cell lymphoma resemble EPF.     

Eosinophilic pustular folliculitis: A review of the Japanese published works

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 non‐steroidal 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.     

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.     

Expression of neuronal nitric oxide synthase in dermal infiltrated eosinophils in eosinophilic pustular folliculitis

Nitric oxide (NO) is produced by a wide variety of human cells and affects physiological and pathophysiological processes. In this study, we demonstrated for the first time that neuronal NO synthase (NOS) is expressed in eosinophils infiltrating into the dermis and follicular epidermis in eosinophilic pustular folliculitis (EPF). EPF is a cutaneous inflammatory follicular disorder first described in Japan. The clinical and histological features of EPF are characterized by erythematous papules, infiltration of numerous eosinophils, and a spongiotic appearance of the follicular epidermis, but the pathophysiology of EPF remains unclear. Our results suggest that NO produced from eosinophils plays an important part in the pathogenesis of EPF. Furthermore, we speculate that NOS inhibitors may be useful in the management of EPF.     

原发性癫痫伴发嗜酸性脓疱性毛囊炎1例

患者,女性５４岁,有４０年原发性癫痫病史,长期服用卡马西平等抗癫痫药物。皮肤损害表现为面部、躯干、上肢环形扩展的红色斑块,其上可见有一致性丘疹,顶部有脓疱。组织病理变化见表皮及毛开口处为嗜酸细胞性脓肿,毛囊深部及周围有局灶性嗜酸细胞为主的炎细胞浸润。ＰＡＳ阴性。认为本病的发生与卡马西平有一定的关系。     

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.     

Eosinophilic pustular folliculitis (Ofuji's disease) in a patient with silicone tissue augmentation

Eosinophilic pustular folliculitis (EPF) (Ofuji's disease) is a rare dermatosis of unknown etiology. We describe a 45-year-old Chinese woman who developed EPF on her face seven years after having nose and chin augmentation with subcutaneous silicone injections.     

Wells' syndrome (eosinophilic cellulitis)--case report and electron microscopic studies

We report a case of Wells' syndrome (eosinophilic cellulitis) with acute cutaneous swelling followed by indolent infiltration. The histopathology is characterized by a dense infiltrate of eosinophils and "flame figures" in the dermis. The electron microscopic findings are peculiar. This case responded well to oral antihistamines. The skin signs and symptoms disappeared completely two weeks later.     

Eosinophilic pustular folliculitis: the first case associated with hepatitis C virus

Eosinophilic pustular folliculitis is a rare disorder that is characterized by recurrent crops of papules and pustules localized on seborrheic areas of the body. The etiopathogenesis of the disease is still unknown, but it has been related to infections and immunosuppressive states. This report is the first case to be found to be related to hepatitis C virus infection.     

Recalcitrant eosinophilic pustular folliculitis of Ofuji with palmoplantar pustulosis: dramatic response to narrowband UVB phototherapy

Eosinophilic pustular folliculitis of Ofuji is a recalcitrant disease typified by non‐infective eosinophilic spongiosis involving the infundibular region of the hair follicle. We present a case of a 49‐year‐old Chinese man with known palmoplantar pustulosis and acrodermatitis continua of Hallopeau which was promptly resolved with methotrexate therapy. He returned with an erythematous papulopustular eruption with coalescence to annular plaques, occurring over the face, chest and back with active palmoplantar pustulation. Histology from skin biopsy of the palmar lesion was in keeping with palmoplantar psoriasis, while biopsy of the facial and truncal lesions revealed florid perifollicular eosinophilic congregation diagnostic of eosinophilic pustular folliculitis of Ofuji. Indomethacin was initiated with partial improvement of lesions with cyclical flares. A trial of narrowband ultraviolet‐B phototherapy at a frequency of thrice weekly achieved sustained clearance of both eosinophilic pustular folliculitis and palmoplantar lesions. Indomethacin was tailed down and eventually discontinued with maintenance of narrowband ultraviolet‐B therapy; this achieved successful control of the disease.     

Eosinophilic pustular folliculitis: report of seven cases in Thailand

Seven cases of eosinophilic pustular folliculitis (EPF) were diagnosed at the Institute of Dermatology within the seven years from 1989-1996. There were 4 males and 3 females, age 24-44 years, with durations of the disease before diagnosis ranging from 3 months to 5 years. The face was the most commonly involved area. Lesions also occurred simultaneously on other sites including the chest, trunk, scalp, and palmoplantar areas. Diagnosis was made from clinical features, hematologic examinations, and histopathologic pictures. Topical piroxicam gel, 0.5%, and oral indomethacin gave good results; some lesions subsided within a few days, and the remissions lasted for four months to five years.