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.     

Eosinophilic pustular folliculitis following autologous peripheral blood stem-cell transplantation

We describe a case of eosinophilic pustular folliculitis (EPF) occurring after autologous peripheral blood stem-cell transplantation for non-Hodgkin's lymphoma and successfully treated with oral and topical indomethacin. Including ours, the reported cases of EPF with HIV-negative hematological disease have shown clinical features similar to those of HIV-associated EPF, however the prognosis is better than that for HIV-associated EPF, which usually shows a persistent course. EPF with HIV-negative hematological disease can be classified as a unique group in EPF.     

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 starting initially only with palmoplantar pustular lesions. Report of a case and review of the literature.

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.     

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.     

Eosinophilic pustular folliculitis (Ofuji's disease) without macroscopic pustules

Background Eosinophilic pustular folliculitis (EPF), first described by Ofuji et al. in 1970, is a rare dermatosis characterized by pruritic papules and pustules on circinate erythematous plaques with eosinophilic infiltration in and around the hair follicle. Methods and results We report three cases of EPF that showed no macroscopic pustules during the total observation period. Histopathological examination revealed eosinophilic infiltration in and around the hair follicle in the lower dermis and subcutis. Conclusions EPF consists of clinical variants, one of which lacks obvious pustules.     

HIV-associated eosinophilic folliculitis in an infant.

HIV-associated eosinophilic folliculitis (HIV-EF), which is a well-known entity in adults, has not been described in children. Although Ofuji's disease (OD) or eosinophilic pustular folliculitis (EPF) has been described in children and shares histopathologic features with HIV-EF, it is a distinct entity with characteristic clinical features. We report the occurrence of eosinophilic folliculitis in an 8-month-old HIV-positive patient and discuss the clinical, pathologic and possible pathogenetic aspects thereof. In addition, differences in the clinical manifestations of the present case and that of I-EPF are addressed. Because of clinicopathologic similarities between the present case and HIV-associated eosinophilic folliculitis (HIV-EF) in adults, we believe that eosinophilic folliculitis in this patient represents a cutaneous manifestation of HIV infection, rather than co-incidental occurrence of the infantile form of Ofuji's disease in an HIV-positive patient.     

Two cases of eosinophilic pustular folliculitis associated with parasitic infestations

Two patients presented with papular eruptions that could not be diagnosed clinically. Biopsies of these lesions showed eosinophilic pustular folliculitis (EPF). Both cases were subsequently found to have infestations: one as a result of cutaneous larva migrans was successfully treated with ivermectin and the other caused by scabies mites was successfully treated with topical pyrethrin. The two cases of EPF presented emphasize the need for careful clinicopathological correlation to determine the cause.     

Eosinophilic pustular folliculitis (Ofuji's disease) and non-Hodgkin lymphoma.

The authors report the third case of eosinophilic pustular folliculitis (EPF) associated with a non-Hodgkin lymphoma. The dermatosis occurred after an autologous bone marrow transplantation performed as treatment for the lymphoproliferative disorder. Although EPF was initially described as an idiopathic disease, the association of some cases with immunologic alterations or diseases, such as immunodeficiencies, suggests a possible immunopathologic event in the pathogenesis of EPF.     

嗜酸性脓疱性毛囊炎1例及临床分析

目的了解中国人群嗜酸性脓疱性毛囊炎(EPF)的临床特点。方法报道1例男性嗜酸性脓疱性毛囊炎患者并对近10年来中国学者报道的20例患者做一总结分析。结果 20例患者中,男19例,女1例;发病年龄为20～50岁的中青年人群;发病部位以头面部为主,其次为躯干、四肢,掌跖部位受累少见;除2例艾滋病患者外,其他患者未伴发任何其他疾病;15例患者外周血嗜酸性粒细胞升高,5例不升高,比例为3:1。结论 EPF发病以男性为主,发病部位及临床表现存在一定差异,外周血嗜酸性粒细胞可升高或不升高。     

Eosinophilic pustular folliculitis (Ofuji disease) manifested as pustules on the palms and soles

Eosinophilic pustular folliculitis (EPF) is clinically characterized by eosinophilic follicular papulopustules that form annular configurations on the face, trunk, and extremities. We describe a case of a patient whose EPF manifested on the soles of the feet, an area that normally does not contain hair follicles. The patient experienced a dramatic therapeutic response to indomethacin.     

A Case of Eosinophilic Pustular Folliculitis (Ofuji's Disease) Induced by Patch and Challenge Tests with Indeloxazine Hydrochloride

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 (Ofuji's disease) in Singapore: a review of 23 adult cases

The case records of 23 patients with classic eosinophilic pustular folliculitis (EPF), or Ofuji's disease, seen at the National Skin Centre in Singapore, from 1990 to 2001 were reviewed. All patients had clinical and histopathological findings consistent with EPF. There were eight men and 15 women (ratio 1:1.6). The mean age at presentation was 35 years. There was a marked predilection for Chinese patients (87%), with a racial distribution of 20:2:1 of Chinese, Malay and Indian patients, respectively. The most frequent site of occurrence was the face, particularly over both cheeks. The majority of patients (90%) treated with oral indomethacin had a good response within 2-4 weeks. Relapses were frequent in 82.6% of patients and maintenance with indomethacin or ketoprofen was beneficial. Eosinophilic pustular folliculitis is a rare but important disease entity presenting with recurrent indurated erythematous papulopustules and plaques on the face. Increased awareness of this condition is important as it can mimic many other conditions presenting as red plaques on the face.     

Eosinophil-infiltrating folliculitis in childhood--report of a case

A 5-year-old Japanese girl developed follicular pustules with erythematous plaques on the lower extremities. The histopathology was compatible with eosinophilic pustular folliculitis (EPF). However, the clinical findings were not typical of EPF; the pustules did not appear in crops, the pustules became ulcers, and the skin lesions appeared exclusively on non-seborrheic areas.     

Eosinophilic pustular folliculitis (Ofuji's disease): efficacy of isotretinoin

The case of a 30-year-old man with a 6-year history of eosinophilic pustular folliculitis (EPF) is reported. Isotretinoin (1 mg/kg/day) led to a dramatic improvement of all the lesions within 2 weeks. The withdrawal of the drug was followed by a recurrence after 10 days of the papulopustular, follicular and pruritic lesions. Reintroduction of isotretinoin was successful. The benefits of isotretinoin in the treatment of EPF have, to the best of our knowledge, never been reported previously. The mechanisms underlying this efficacy may involve the inhibition of the eosinophilic chemotactic factors thought to be present in sebaceous lipids and in the stratum corneum of patients suffering from EPF.     

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

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.     

Eosinophilic pustular folliculitis in infancy: not a distinctive inflammatory disease of the skin

In the standard literature of dermatology and dermatopathology "eosinophilic pustular folliculitis in infancy" is presented as a distinctive inflammatory disease of the skin, to wit, a variant of "Ofuji's eosinophilic folliculitis". Assessment critically of the first publication devoted to the subject revealed a potpourri of findings clinical and histopathologic, with no clear criteria being set forth by the authors to enable diagnosis, with precision. Our review of all articles dedicated to the matter of "eosinophilic pustular folliculitis in infancy" shows that criteria for diagnosis are found with great difficulty. The majority of patients reported on had neither "eosinophilic folliculitis," nor did they present themselves clinically in any way similar to what was originally described by Ofuji. The findings histopathologic told of most commonly were dense and diffuse infiltrates in the dermis that contained many eosinophils, sometimes with periadnexal distribution. We think that the findings reported on in several articles suggest that the patients more likely had a variety of different diseases, among them scabies, insect bites, impetigenized nummular dermatitis, and linear IgA-dermatosis. For clarification of terminology, we suggest that "eosinophilic folliculitis" is better defined as a pattern histopathologic than as a distinctive disease entity. It may be encountered in a variety of conditions (eg, Ofuji's disease, arthropod bites, scabies, or dermatophytosis). In sum, no clear criteria have been established for diagnosis of "eosinophilic pustular folliculitis of infancy" and there is no convincing evidence, at present, that "eosinophilic pustular folliculitis of infancy" qualifies as a distinctive inflammatory disease of the skin.     

Successful treatment of eosinophilic pustular folliculitis with topical tacrolimus 0.1 percent ointment

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.     

Eosinophilic Pustular Folliculitis (Ofuji's Disease) in Myelodysplastic Syndrome

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.