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We describe a case of eosinophilic pustular folliculitis (EPF, Ofuji's disease) in a 12-year-old male who suffered from myelodysplastic syndrome. Bone marrow study revealed an increase in the eosinophil cell line without peripheral blood eosinophilia in our case. We suggest that the immunologic abberations ascribed to myelodysplastic syndrome and the increase in the eosinophil cell line in the bone marrow might play roles in the development of EPF in our case.

We report the successful treatment with ultraviolet B phototherapy of a patient with HIV-associated eosinophilic pustular folliculitis. We were able to observe the clinical and therapeutic course for about one year and three months. This 35-year-old homosexual Japanese man presented with disseminated, discrete, follicular, erythematous papules with intense pruritus over his face, neck, chest wall, and upper back. Initially, the eruption responded to therapy with topical or oral indomethacin and oral H1 antihistamine. However, the eruption was highly prone to recurrence, and it gradually failed to respond to these therapies. The eruption became chronic and persistent and manifested the excoriated, prurigo-like nodules that are typical of reported pruritic papular eruption, suggesting that this skin disease and HIV-associated eosinophilic pustular folliculitis are two forms of the same disease entity. UVB phototherapy in small doses was very effective for the persistent eruption, and no recurrence of the eruption was noted during or since the six-month maintenance therapy (once a week at a dose equivalent to 0.75 of the minimal erythema dose) (9 months total). No unfavorable side effects have been observed during or after the UVB phototherapy (cumulative UVB doses of 2,320 mJ/cm2).

Interferon-γ is a multifunctional cytokine produced by Th1 cells. We have observed clinical efficacy of this cytokine in the treatment of Ki-1+ lymphoproliferative disorders, eosinophilic pustular folliculitis and chronic prurigo.

Childhood eosinophilic pustulosis is a rare disease that is characterized by recurrent outbreaks of pruritic pustules and follicular papules. The lesions are sterile and contain masses of eosinophils related to the scalp hair follicle. Because of the good prognosis for these symptoms, conservative treatment with topical corticosteroids is recommended. We present two cases of this disease, describing its clinical course and development over two years.

Objective: We present a case of eosinophilic pustular folliculitis, a rare dermatosis which is often associated with HIV infection or internal malignancies. Clinical Presentation and Intervention: We report the case of a 66-year-old man with a medical history of hypertension. Histopathological examination showed a dense follicular inflammatory infiltrate with abundant eosinophils. The clinical response to indomethacin was excellent with no recurrence during the follow-up. Conclusion: The patient responded well to indomethacin treatment.

Summary Background Although more than 300 cases of eosinophilic pustular folliculitis (EPF) have been reported to date, differences in clinicohistopathological findings among affected sites have not yet been evaluated. Objectives To evaluate differences in the clinical and histopathological features of facial and extrafacial EPF. Methods Forty-six patients diagnosed with EPF were classified into those with facial and extrafacial disease according to the affected site. Clinical and histopathological characteristics were retrospectively compared, using all data available in the patient medical records. Results There were no significant between-group differences in subject ages at presentation, but a male predominance was observed in the extrafacial group. In addition, immunosuppression-associated type EPF was more common in the extrafacial group. Eruptions of plaques with an annular appearance were more common in the facial group. Histologically, perifollicular infiltration of eosinophils occurred more frequently in the facial group, whereas perivascular patterns occurred more frequently in the extrafacial group. Follicular mucinosis and exocytosis of inflammatory cells in the hair follicles were strongly associated with facial EPF. Conclusions The clinical and histopathological characteristics of patients with facial and extrafacial EPF differ, suggesting the involvement of different pathogenic processes in the development of EPF at different sites.

First described in Japan by Ofuji in 1970 [1], eosinophilic pustular folliculitis (EPF) is a noninfectious inflammatory skin disease that manifests with coalescing papulopustular plaques. The disease is histologically characterized by eosinophilic infiltration of hair follicles. There are three known variants of EPF: classic (as originally described by Ofuji and predominantly affecting Japanese individuals), HIV-associated, and infantile.

Eosinophilic pustular folliculitis (EPF) is a disorder associated with a high expression of interleukin-5 by T helper 2 cells. Treatment involving T helper 1 (Th1) modulation has been shown to be effective. We report that the occurrence of Bowen's disease in the medical care zone of the University of Occupational and Environmental Health in the Kitakyushu industrial area is more frequent in Yahatahigashi-ku, Yahatanishi-ku, and Wakamatsu-ku than in Tobata-ku, Kokurakita-ku, and Kokuraminamiku. We also show that these cases are more common in the regions with steel- and coal-related industries, which is suggestive of a higher rate of Th1 modulation associated with these occupations. Similarly, the incidence of EPF per unit population was found to be high in Tobata-ku and low in Yahatahigashi-ku, which indicates that EPF is a typical disease of hygiene hypothesis.

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.

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.

A 73-year-old male developed disseminated erythema over his entire body after exposure to indeloxazine hydrochloride, a cerebral activator. Patch testing with indeloxazine hydrochloride showed a positive reaction, and plaques, vesicles and pustules developed on the face after the patch test. These had the pathologic feature of eosinophilic pustular folliculitis (EPF, Ofuji's disease). A challenge test also provoked eruptions on the face, trunk, arms and legs, which were compatible with EPF. Moreover, both the patch and challenge tests with indeloxazine hydrochloride induced eosinophilia. This is the first report of drug allergy-induced EPF, where drug sensitivity induced an abnormal eosinophilic response mimicking EPF.

Eosinophilic pustular folliculitis (EPF) is characterized clinically by pruritic grouped follicular papules and pustules on the trunk, limbs, and face, and, histologically, by follicular infiltration with eosinophils. The blood eosinophil count is elevated in most patients. Oral minocycline, nonsteroidal anti-inflammatory drugs, diaminodiphenylsulphone, and corticosteroids may induce remission. We report two Japanese men with EPF who responded poorly to the usual therapy. Intravenous injections of recombinant interferon-gamma (rIFN-gamma), 5 x 10(5) to 2 x 10(6) Japan Reference Unit (JRU) (1 JRU roughly corresponds to 4 NIH units) daily for 7 days, cleared the skin lesions and returned the peripheral eosinophil counts to normal in both patients. However, the lesions recurred 2-3 days after rIFN-gamma was stopped. Both patients have received intravenous rIFN-gamma once or twice a week for nearly 1 year without systemic side-effects. Reverse transcriptase-polymerase chain reaction revealed a decreased expression of interleukin 5 (IL-5) mRNA in peripheral mononuclear cells after the rIFN-gamma therapy. rIFN-gamma may become the treatment of choice in recalcitrant EPF, although further studies are needed. It may work by interfering with the immunological function of type 2 T-helper cells, including IL-5 production responsible for the growth and differentiation of eosinophils.

We report human immunodeficiency virus (HIV)-associated eosinophilic folliculitis in a previously well 40-year-old Portuguese lady. This condition, although similar to Ofuji's disease, has distinguishing features which allow the two to be differentiated. To the best of our knowledge, this is the first reported case of HIV-associated eosinophilic folliculitis in a female and in a patient in whom the HIV was heterosexually transmitted. Our patient is also unusual in being, we believe, only the second reported in whom this eruption was the presenting feature of HIV infection.

30歳の男性。掌蹠に紅斑が初発し病初期は異汗性湿疹, その後膿疱が新生したことより掌蹠膿疱症と診断されていたが, 体幹に皮疹が拡大した時期の病理組織学的所見より好酸球性膿疱性毛嚢炎の診断にいたった症例を経験した。インドメサシン, 塩酸ミノサイクリン内服にて膿疱は消失し紅斑も軽快した。また, これまで当科で経験した好酸球性膿疱性毛嚢炎の22例について集計した。最近経験した1症例についてweak androgenを測定したところ高値を示した。治療経過においてインドメサシン内服が最も有効で, 大部分の症例で内服開始後数日で皮疹は改善した。しかし減量, 中止の過程で殆どの症例が再燃し, 数ヵ月から数年症状が持続している。

A 20-year-old lady presented with a 3-month history of an itchy eruption affecting her face and trunk. Erythematous annular plaques with follicular papules and pustules at the margins were present on her face consistent with Ofuji's disease. A perifollicular and perivascular eosinophil-rich infiltrate was seen histologically, with infiltration of the follicular epithelium by eosinophils and formation of eosinophilic microabscesses. An unusual feature was the presence of follicular mucinosis. Treatment with dapsone resulted in an improvement in the eruption after 2 weeks. Ofuji's eosinophilic pustular folliculitis is becoming increasingly frequently recognized, and may not be as rare as initial reports suggested. In 1965, Ise and Ofuji reported the case of a Japanese woman who had recurrent episodes of follicular pustules affecting her face and back accompanied by a peripheral eosinophilia. Following this, in 1970 Ofuji et al. reported three further cases and proposed the name eosinophilic pustular folliculitis. Although many of the early cases were in Japanese patients, reports of this disease affecting individuals from Europe and the United States have been increasing.

We describe a patient with eosinophilic pustular folliculitis who partially responded to oral indomethacin, but intermittently experienced new eosinophilic pustular folliculitis lesions. Treatment with tacrolimus ointment 0.1% resulted in the rapid improvement of each recurred lesion and allowed withdrawal of indomethacin. To our knowledge, this is the first report of the use of topical tacrolimus in the treatment of eosinophilic pustular folliculitis.

Dargestellt wird ein Fall einer bei Menschen nicht japanischer Herkunft nur selten beobachteten sterilen eosinophilen Pustulose Ofuji (SEP). Das distinktive histologische Bild mit intraepidermalen eosinophilenreichen Abszessen und eosinophiler Follikulitis wird erganzt durch eine ausgepragte Bluteosinophilie, eine deutliche eosinophile Knochenmarksreaktion und den offenbar erstmalig beobachteten Mundschleimhautbefall mit histologischem Nachweis eosinophiler Spongiose. Mogliche Ubergange zwischen kutanen und internen Hypereosinophilie-Syndromen werden diskutiert. Trotz des zunachst guten Ansprechens der kutanen Veranderungen auf topische Kortikosteroide ist eine Langzeituberwachung hinsichtlich interner Mitbeteiligung notwendig.

Three children will be described who present recurrent episodes of pruritic papulopustular follicular lesions on the face, the extremities and the trunk. The episodes lasted for 1-3 months with intermittent remission. Each flare was accompanied by hypereosinophilia and an increased total IgE titer. RAST and prick tests were positive for Dermatophagoides pteronyssinus (DPT). Laboratory tests disclosed no infectious or parasitic etiology. Histological examination showed eosinophilic pustular folliculitis (EPF) in each of the 3 cases. The lesions responded well to topical corticosteroids. The aim of this article is to underline the importance of hypersensitivity reactions (in these particular cases to DPT) in the pathogenesis of EPF.

We report a new case of eosinophilic pustular folliculitis in a 23-month-old boy. He presented with a seven-month history of recurrent episodes of pustular lesions on the scalp after having been treated with oral antifungal and topic antibiotics without response. The diagnosis was based on the clinical course and typical histopathological findings. Eosinophilic pustular folliculitis in infancy is an idiopathic and rare inflammatory disease characterized by recurrent crops of sterile pustules involving mainly the scalp. Because it is a benign, self-limiting condition an accurate diagnosis is essential to avoid unnecessary therapies.

Eosinophilic pustular folliculitis is a pruritic eruption that preferentially involves the face. It is characterized by well-demarcated erythema, extending peripherally with a central clearing and pigmentation, together with sterile pustules lining the periphery. We describe five cases of eosinophilic pustular folliculitis with pruritic papules and erythema on seborrheic areas of the face, which lacked the typical features of classic eosinophilic pustular folliculitis--pustules and peripheral extension--but showed eosinophilic infiltration of the hair follicles, histologically. The eruption quickly responded to oral indomethacin except for one case that responded to tranilast and one case that was associated with acquired immunodeficiency syndrome, with recurrences in defined areas of the face. Our findings in these cases suggest that eosinophilic pustular folliculitis may vary in clinical appearance.

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.

Classic eosinophilic pustular folliculitis (EPF), otherwise known as Ofugi disease, is a rare condition commonly treated with topical glucocorticosteroids. If this fails, oral indomethacin is frequently the next line. Because the condition is recurrent, the use of long term steroids may cause side effects such as skin atrophy, hypertrichosis, and dyspigmentation. Topical tacrolimus is an immunosuppressant that is generally used as a steroid-sparing agent in atopic dermatitis. We report a case of classic EPF, which was recurrent over 5 years that had failed topical glucocorticosteroids but was successfully treated with topical tacrolimus 0.1 percent ointment.

The report deals with two cases of eosinophilic pustular folliculitis in a 45-year-old man and a 25-year-old woman. After their conditions failed to respond to oral and topical corticosteroids, minocycline, anti-allergic drugs, aspirin and several types of nonsteroidal anti-inflammatory drugs, good results were obtained with acemetacin.

A 25-year-old female had a 4-month history of bilateral cervical lymphoadenopathies, weight loss, mild fever, and severe pruritus. A few months earlier, allergological analysis had been performed which revealed raised IgE (4.6 kU/L) against fungi antigens. A right cervical node biopsy led to histological diagnosis of nodular sclerosis-type Hodgkin lymphoma. Clinical symptoms, bilateral cervical lymphoadenopathies, and a large mediastinal involvement (revealed by chest X-ray and thoracic TC) were characteristic of stage IIB (bulky disease). The patient was treated with adriblastine, bleomycin, vinblastine, dacarbazine polychemotherapy (ABVD). Immediately after the administration of this cycle, she developed slightly erythematous, follicular, itchy papules and pustules, localized on her scalp (Fig. 1), trunk (Fig. 2), and thighs. A biopsy from the scalp was obtained under local anesthesia, after patient's consent. Histopathologic findings were characterized by a superficial and deep, dense perifollicular and interstitial infiltrate composed mainly of eosinophils, accompanied by few neutrophils and lymphocytes. Eosinophilic folliculitis involved the whole follicle (inferior segment, isthmus and infundibulum), and also the sebaceous gland (Fig. 3). PAS revealed the presence of some spores of Pityrosporum on the epidermis and also in the follicle. Gram stains proved negative. Eosinophilic folliculitis in an immunocompromised patient was diagnosed. Laboratory findings ascertained that the eosinophil count was in the normal range (eosinophils: 1%; white blood cell count: 10 700/mm3). The lesions healed spontaneously in about 10 days. After a second cycle of polychemotherapy (without dacarbazine, because the patient suffered repeated vascular injection injuries due to this chemotherapeutic agent), new crops of lesions in the same areas developed and in a few days faded away spontaneously. The blood clotting analysis performed just before this new cycle of ABV therapy revealed an eosinophil count of 10%, with a white blood cell count of 4500 mm3. Radiotherapy and four additional cycles of ABV polychemotherapy were made; in the meanwhile, the patient did not develop cutaneous lesions. Actually she is free from Hodgkin lymphoma and from eosinophilic folliculitis. Figure 1. Pustules with extrusion of whitish material, on the patient's scalp Download figure to PowerPoint Figure 2. Papules and pustules, some of them excoriated, localized on the chest Download figure to PowerPoint Figure 3. The eosinophilic infiltrate is present also inside the sebaceous gland, a typical finding of eosinophilic folliculitis in immunocompromised patients (H&E, original magnification × 40) Download figure to PowerPoint

Background Eosinophilic pustular folliculitis (EPF) is a chronic intractable pruritic dermatosis characterized by massive eosinophil infiltrates involving the pilosebaceous units. Recently, EPF has been regarded as an important clinical marker of HIV infection, and its prevalence is increasing in number. The precise mechanism by which eosinophils infiltrate into the pilosebaceous units remains largely unknown. Given that indomethacin, a COX inhibitor, can be successfully used to treat patients with EPF, we can assume that COX metabolites such as prostaglandins (PGs) are involved in the etiology of EPF. Objective To determine the involvement of PGs in the pathogenesis of EPF. Methods We performed immunostaining for PG synthases in EPF skin lesions. We examined the effect of PGD 2 on induction of eotaxin, a chemoattractant for eosinophils, in human keratinocytes, fibroblasts, and sebocytes and sought to identify its responsible receptor. Results Hematopoietic PGD synthase was detected mainly in infiltrating inflammatory cells in EPF lesions, implying that PGD 2 was produced in the lesions. In addition, PGD 2 and its immediate metabolite 15-deoxy-Δ 12,14-PGJ 2 (15d-PGJ 2 ) induced sebocytes to produce eotaxin-3 via peroxisome proliferator–activated receptor gamma. Consistent with the above findings, eotaxin-3 expression was immunohistochemically intensified in sebaceous glands of the EPF lesions. Conclusion The PGD 2 /PGJ 2 -peroxisome proliferator–activated receptor gamma pathway induces eotaxin production from sebocytes, which may explain the massive eosinophil infiltrates observed around pilosebaceous units in EPF.

This study reports on the clinical and light microscopic features of a nevoid basal cell carcinoma syndrome with the complication of eosinophilic pustular folliculitis. To the authors' knowledge, this is the first report of such an association, which is possibly due to immune dysregulation. Moreover, the patient experienced remission of eosinophilic pustular folliculitis after removal of the jaw cyst. One possible explanation for the remission is that a long-lasting TH, type inflammatory response as a result of the bone defect produces effective cytokines such as interferon-gamma.

Eosinophilic pustular folliculitis (EPF) is characterized by erythematous patches of large follicular papules and pustules involving mainly the face. Although various treatments have been attempted for EPF, including systemic and topical steroid, diaphenylsulphone, colchicine, minocycline as well as UVB phototherapy, there is no consensus on the first choice of treatment. We report a typical case and summarize 25 patients with EPF treated in our hospital between 1978 and 1998. Indomethacin was most frequently used (12/25) and showed clinical improvement in the majority of the cases (11/12). The effect of indomethacin was usually observed within 1--2 weeks after initiation of treatment. Decrease of peripheral blood eosinophils accompanied the clinical improvement. Thus, indomethacin should be considered as a first choice of treatment for EPF.

The complaint of itch is common in patients with AIDS and HIV infection. Inspection of the skin often reveals a dermatosis, but precise diagnosis is not always easy which makes it difficult for effective treatment. In HIV-positive patients with itch, as well as common dermatoses, one often has to deal with atypical and even new skin diseases. Many of these are papular, papulo-vesicular or papulo-pustular. This article is a protocol for the diagnosis and treatment of itch in these patients.

Background: Among sterile pustuloses in childhood, a pruritic relapsing eosinophilic variant beginning in infancy and located mostly in the scalp was first described as eosinophilic pustular folliculitis in infancy by Lucky and colleagues in 1984. Objective: Our purpose is to describe such a condition in five boys and one girl and comment on differential diagnosis and relation with Ofuji's disease. Method: This is a clinicopathologic study. Results: All patients had scalp pustules beginning in infancy or early childhood that were unresponsive to antibiotic therapy. Lesions also occurred on other areas but the scalp was the major site of involvement. Although secondary infection was demonstrated in one case, the lesions were primarily sterile. Smears of pustules showed a variable proportion of eosinophils. Histopathologic findings suggested a major role for eosinophils in this disorder because dermal eosinophilia was noted in all patients. The inflammatory pattern was not similar to Ofuji's disease. Transient blood eosinophilia was recorded in five patients. Topical steroids relieved inflammatory episodes. Dapsone was tried in one case with apparent benefit. Conclusion: Eosinophilic pustulosis of the scalp in childhood is a self-limited disease that can be relieved by topical steroids.

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.

We report a 23-year-old Japanese male with eosinophilic pustular folliculitis (EPF) that had started with palmoplantar rash. Only when follicular pustules appeared on the bilateral cheek 31 months later, we revised our initial diagnosis of pustulosis palmaris et plantaris (PPP) to EPF, and all the skin eruptions cleared mostly with indomethacin. A review of the Japanese literature for the past 20 years disclosed that in 207 cases of EPF so far reported, palmoplantar pustular lesion was noted in 38 (18%). Among them, in 16 cases (8%) the skin lesions started first from the palmoplantar region with the average period of 26 months until the appearance of other eruptions of EPF. None of them was diagnosed as EPF when skin lesions were localized only to the palmoplantar region. When we find pustules on the palmoplantar region, we should suspect the possibility of early lesions of EPF as well as PPP. Histopathologic demonstration of multilocular pustules located in the upper epidermis containing numerous eosinophils in the palmoplantar pustular lesions, together with the dramatic therapeutic response to indomethacin greatly favor the diagnosis of EPF.

A 37-year-old man was first seen in November 1998 with a unilateral painful eruption of grouped small vesicles at the right side of his thorax. His general health was good. He was diagnosed as having herpes zoster, which was successfully, treated with oral acyclovir 800 mg, five times a day. Five months later, and after complete resolution of the herpes zoster, he developed a pustular eruption on exactly the same area of his first herpetic lesion. There was a diffuse distribution of pustules on the dorsal part of the dermatome, and a grouped pattern on the ventral side ( Fig. 1). A punch biopsy was performed for differential diagnosis of recurrent herpes zoster and folliculitis. Methicillin-resistant Staphylococcus aureus colonies were isolated from the bacterial culture of the pustular content. Cefadroxil monohydrate 500 mg twice a day and the application of fucidic acid ointment were prescribed. There was no improvement at the end of the second week of therapy. Figure 1. Clinical appearance Download figure to PowerPoint A histopathologic study demonstrated hyperkeratosis, acanthosis, focal accumulation of neutrophils and bacteria in the epidermis, and a perivascular and perifollicular infiltration of eosinophils, lymphocytes, and histiocytes in the dermis ( Fig. 2). After the diagnosis of eosinophilic pustular dermatosis was histopathologically confirmed, fungal folliculitis and immunosuppressive conditions were investigated. Routine blood tests were within the normal ranges. The human immunodeficiency virus (HIV), hepatitis C virus (HCV), and anti-hepatitis B surface antibody (anti-HBs) tests were negative, but the hepatitis B surface antigen (HBsAg) test was positive. Abundant fungal hyphae were seen in potassium hydroxide under direct microscopic examination of the pustule content, and a fungal culture was performed. Although no evidence of fungal infection was demonstrated in repeated periodic acid–Schiff (PAS) staining of punch biopsy specimens, Trichophyton rubrum was isolated from fungal culture. The patient was then given a regimen of itraconazole pulse therapy (400 mg/day, for 7 days a month, repeating the monthly cycle three times) and cycloproxolamine cream. Two months later, at the end of therapy, only a few pustules remained, and these completely disappeared after another 5 months. Figure 2. Eosinophilic pustular folliculitis: dense eosinophilic infiltration within and around the hair follicle (hematoxylin and eosin, × 400) Download figure to PowerPoint

Eosinophilic pustular folliculitis is a rare dermatosis. Recently, in addition to oral indomethacin, other treatments have been applied for eosinophilic pustular folliculitis. The aim of this study was to assess the effectiveness of various therapies encompassing conventional to newly applied drugs for eosinophilic pustular folliculitis. Twenty patients with eosinophilic pustular folliculitis seen in our department were investigated. The effectiveness of each treatment was assessed by a severity score index. Eleven patients were treated with oral indomethacin, and the severity scores of all patients were decreased after the treatment. Oral cyclosporine was markedly effective in all 11 patients treated, and topical tacrolimus ointment alleviated eosinophilic pustular folliculitis in 3 of 7 with one patient showing a remarkable reduction in the severity score. In addition to indomethacin or other oral non-steroidal anti-inflammatory drugs, oral cyclosporine and topical tacrolimus may be beneficial choices when patients have been resistant to previous treatments.

Eosinophilic pustular folliculitis is characterized by the spontaneous development of recurrent, sterile papules, pustules, and plaques on the face, trunk, arms, and occasionally the palms and soles. Although the large majority of the reported cases have occurred in Eastern Asians, most patients in the United States have been infants or men seropositive for human immunodeficiency virus. We describe a North American woman with eosinophilic pustular folliculitis who was neither seropositive for human immunodeficiency virus nor of Asiatic descent.

Dear Editor: We report a case of generalized eosinophilic pustular folliculitis of infancy (EPFI) involving the scalp, face, trunk, and both lower extremities that was treated with a topical corticosteroid and hydroxyzine. Interestingly, the lesions responded to hydroxyzine in a dose-dependent manner. A 3-month-old female infant presented with itchy erythematous papules and pustules on the face, trunk, and legs that had been present since birth (Fig. 1A~C). We performed a routine blood test and skin biopsy on a pustule of the trunk. Laboratory tests showed eosinophilia (eosinophil count: 5.19×109/L, normal range: 0.05~0.45×109/L), and histopathologic examination showed subcorneal pustule formation, and perivascular lymphohistiocytic and eosinophilic infiltration (Fig. 2). Therefore, a diagnosis of EPFI was made, and she was treated with topical methylprednisolone aceponate 0.1% ointment twice daily and oral hydroxyzine 10 mg/day. Her symptoms and lesions improved greatly within 3 weeks. However, she showed aggravation during hydroxyzine withdrawal; when hydroxyzine treatment was consequently resumed, the lesions improved dramatically (Fig. 1D~F). Eosinophil count decreased to 0.73×109/L 3 months later. Fig. 1 (A~C) Crops of papules and pustules on the face, chest, and pubic area at presentation. (D~F) After treatment with oral hydroxyzine, the skin lesions resolved almost completely without scarring. Fig. 2 Subcorneal pustule formation and perivascular lymphohistiocytic infiltration with many eosinophils (HE A: ×50, B: ×200). EPFI, a relatively rare dermatologic disease that develops in early infancy, is usually characterized by recurrent crops of papules and pustules on the scalp and other body areas1. The etiology of EPFI remains unknown but may be associated with some genetic susceptibility because of its male predilection and higher incidence in Caucasians2. Because many reported cases have not demonstrated true follicular involvement, some authors propose the term "eosinophilic pustulosis of infancy" is more suitable2,3. EPFI must be differentiated from other dermatological diseases characterized by recurrent grouped papules and pustules occurring in the neonatal period. However, EPFI develops most frequently in first months of life, and over 80% of patients experience spontaneous resolution by 3 years of age2. Moreover, most cases present with multiple pruritic lesions on the scalp and are associated with tissue eosinophilia or blood eosinophilia. Good response to topical corticosteroids is strongly indicative of EPFI2. Erythema toxicum neonatorum may exhibit similar cutaneous manifestations and histopathologic findings in neonates. However, EPFI may be distinguished from this disease according to its recurrent nature, predilection towards the scalp, and associated blood eosinophilia (83%) during attacks2. There are various treatment options for EPFI, including topical corticosteroids, topical calcineurin inhibitors, and oral antihistamines3. Some refractory cases can be treated with erythromycin3, dapsone3, or indomethacin4, but the effectiveness of these medications varies. EPFI usually responds well to topical corticosteroids2,4 unlike EPF in adults. The present case showed slight improvement after the topical corticosteroid was administered and an excellent response after hydroxyzine treatment was initiated. Oral antihistamines such as cetirizine2 have been successfully used by clinicians; interestingly, the present case presented with lesion fluctuation with respect to the use of hydroxyzine or lack thereof. The generalized form of EPF is reported to be associated with several medications, especially allopurinol5, whereas EPFI is not. However, in patient case, we observed disseminated papules and pustules on nearly the entire body without a previous history of medication. Although the present case is considered to belong to same disease spectrum as the localized form, generalized EPFI developing in a patient without a history of medication has never been reported.

乾癬の経過中に好酸球性膿疱性毛嚢炎(EPF)がみられた1例を報告した。症例は33歳の男性。10年来の乾癬の経過中, 顔面, 背部に膿疱を伴った局面が, 掌蹠に紅斑性角化局面と膿疱が出現した。病理組織像では毛包内に好酸球·好中球からなる多房性膿瘍を, 末梢血ではEPF発症前には認められなかった好酸球増多が認められた。インドメサシン1日50mgを投与後, EPFの皮疹は急速に消退し少数の膿疱が周期的に新生する程度に改善した。一方, 乾癬の典型的皮疹はインドメサシンに反応しなかった。従って自験例では乾癬にEPFが合併した症例と考えた。このような症例の報告は調べ得た限りではみられなかった。

Eosinophilic pustular folliculitis (EPF) is a noninfectious eosinophilic infiltration of hair follicles first described 40 years ago. There are 3 variants: classic EPF, immunosuppression-associated (mostly HIV-related), and infancy-associated EPF. EPF has been classified as an AIDS-defining illness. In both children and adults EPF should be viewed as a possible cutaneous sign of immunosuppression. However, it may also be seen in persons with normal immune status. We review EPF on the 40th anniversary of its original description.

A 15-year-old, unmarried female presented to our dermatology department for an intensely pruritic skin rash that had appeared abruptly 3 days earlier. She had a remarkable medical history for a case of allergic rhinitis and several attacks of asthma in her early childhood. The condition waxed and waned initially but had improved in recent years. Physical examination revealed several erythematous plaques, papules studded with scattered pustules having diameters less than 0.3 mm. Conspicuous scratch marks had caused erythematous wheal-like indurations also studded with pustules in a linear distribution across the waist, forearms (Fig. 1), and back (Fig. 2). Discrete papulopustules were present on the face, nape and neck. The patient was otherwise healthy. There were no other symptoms such as fever, malaise, weakness, or lymphadenopathy Laboratory results were normal for hepatic and renal functions, serum electrolytes, glucose, protein, erythrocyte sedimentation rate (8 mm/h), and C-reactive protein (0.355 mg/l). A human immunodeficiency virus (HIV) antibody screen test was negative. Serum was positive for herpes simplex virus (HSV)-1 and HSV-2 IgG (in low titers), but negative for HSV-1 and HSV-2 IgM. White blood cell count revealed leukocytosis (11.2 x 10(3)/l), with a differential count of 68% neutrophils, 27% lymphocytes, and 8% eosinophils. Serum IgA, IgG, and IgM were within normal limits, but the IgE level was elevated (677 mg/dl). Cultures from peripheral blood and pustules were negative. A Tzank smear performed on the pustules showed no multinucleated giant cells. Fungal testing of skin scrapings from the initial lesion site gave negative results. Routine stool tests, including common pathogen and parasite screens, were negative, and urinalysis results were unremarkable. A biopsy specimen obtained from a skin pustule showed subcorneal eosinophilic and neutrophilic pustules in the follicular infundibulum with marked spongiosis of the follicular epithelium. (Fig. 3). There was a moderately dense superficial and deep perivascular mixed inflammatory cell infiltrate comprising eosinophils, neutrophils and lymphocytes. Migration of eosinophils and neutrophils through the vessel wall with variable luminal intramural fibrin deposition, histologically indicative of vasculopathy, was seen. There was concomitant slight perivascular dermal necrosis. (Fig. 4). Based on the clinical presentation and light microscopic findings on biopsy, a diagnosis of eosinophilic pustular folliculitis with pathergy was made. Systemic prednisolone 30 mg in divided doses was given. After 1 week of systemic corticosteroid therapy, the patient's condition was significantly improved and the patient was subsequently discharged. Two months later she had a relapse, upon which corticosteroid therapy was commenced leading to lesional resolution. The foci of eosinophilic folliculitis healed with areas of hyperpigmentation with variable scarring.

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.

ejd.2013.2117 Auteur(s) : Shuko Okada, Taku Fujimura tfujimura1@mac.com, Sadanori Furudate, Yumi Kambayashi, Katsuko Kikuchi, Setsuya Aiba Department of Dermatology, Tohoku University Graduate School of Medicine, Seiryo-machi 1-1, Aoba-ku, Sendai, 980-8574, Japan Eosinophilic pustular folliculitis (EPF) is a rare dermatitis of unknown etiology, which is reported to correlate with human immunodeficiency virus (HIV) infection [1]. Recent reports suggested that EPF is often accompanied by peripheral [...]