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.     

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.     

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.     

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 (Ofuji's disease): indomethacin as a first choice of treatment

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.     

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.     

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.     

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

Eosinophilic pustular folliculitis (EPF), also known as Ofuji disease, is a disease that manifests with follicular papules or pustules. Its variants include a classic type that occurs most commonly in Japan, an HIV-associated type, an infantile type, a type that occurs on the palms and soles, a rare medication-associated variant, and a rare neoplasia-associated variant.A wide range of medications has been used to treat EPF. Topical corticosteroids are the first-line treatment option for EPF. Topical tacrolimus seems to be useful initial therapy as well. Oral indometacin (50-75 mg/day) is an effective treatment of classic EPF although it can induce peptic ulcers. For treatment of HIV-associated EPF when topical corticosteroids and indometacin do not work, various other treatments should be considered. These treatment options include cetirizine 20-40 mg/day, metronidazole 250 mg three times a day, itraconazole starting at a dosage of 200 mg/day and increasing to 300-400 mg/day, and topical permethrin. If these treatments do not work phototherapy with UVB is the 'gold standard' of treatment and is often curative. Treatments with less certain risk-benefit ratios but with some efficacy include PUVA (psoralen + UVA) photochemotherapy, oral corticosteroids, synthetic retinoids (i.e. isotretinoin 1 mg/kg/day), and acitretin (0.5 mg/kg/day), oral cyclosporine (ciclosporine) 5 mg/kg/day, interferon (IFN)-alpha-2b, and IFNgamma. Minocycline 100mg twice daily and dapsone 50-100mg twice daily have been used with some effect. The use of highly active antiretroviral therapy for HIV has resulted in the amelioration of EPF as CD4 cell counts rise above 250/mm(3). The diversity of clinical presentations and affected populations make it seem that EPF is a reaction pattern as much as a disease and that therapy should be tailored to the variant of EPF and the underlying etiology.     

A distinctive vesiculopustular eruption associated with hepatobiliary disease

Background Cutaneous manifestations of hepatobiliary disease may be a clue to the clinical diagnosis. We encountered a form of vesiculopustular eruption in five patients with hepatobiliary disease. A similar eruption has been described in the setting of ulcerative colitis, and has received the designation of vesiculopustular pyoderma gangrenosum. Methods Skin biopsy specimens were examined by conventional histopathologic methods and, in three cases, fresh tissue was available for direct immunofluorescence examination. A diagnosis of hepatobiliary disease was known in three of the patients and in the other two it was established by the skin biopsies. Results All skin biopsies of the five patients showed a neutrophilic dermatosis that had two morphologies. One was characterised by superficial neutrophilic dermolysis with subepidermal bulla formation and the other by a nonulcerating destructive suppurative folliculitis and perifollicular neutrophilic dermolysis. Both were frequently accompanied by a neutrophilic and lymphotic eccrine hidradermitis and a Sweet's syndrome-like vascular reaction. The lesions were held to represent a novel vesiculopustuiar eruption with features reminiscent of pyoderma gangrenosum. Conclusions We propose the term vesiculopustular eruption of hepatobiliary disease for this distinctive clinicopathologic entity, which bears a strong histologic resemblance to pyoderma gangrenosum. Patients with a known hepatopathy who present with necrotizing vesicles and pustules may have such an eruption. The onset may be the first clue to or herald an exacerbation of underlying liver disease.     

药物所致急性全身发疹性脓疱病11例临床分析

目的：提高临床医师对药物引起急性全身发疹性脓疱病的认识。方法：分析１１例急性全身性发疹性脓疱病的病因、症状、体征、实验室检查和病理,诊断及鉴别诊断。结果：１１例患者发病前均有药物史,临床上均有发热,全身性红斑,小脓疱。病理显示角层下脓疱。结论Ｌ：药物是引起急性全身性发疹性脓疱病的主要原因之一,其药物以抗菌素和解热镇痛类药继主,发热和泛发小脓疱是该病的特点,根据病因,临床表现,诊断并不困难,但需与胞     

Eosinophilic pustule smear in the diagnosis of pediatric post-transplant eosinophilic folliculitis

Hematologic-associated eosinophilic pustular folliculitis is a subtype of eosinophilic pustular folliculitis (EPF) which develops in patients with underlying hematological malignancies after treatment with chemotherapy, bone marrow transplant (BMT), or stem cell transplant (SCT). Few cases of hematological-associated EPF have been reported in pediatric patients. Skin biopsy is considered the gold standard for diagnosis. We describe a case in which Wright staining of a pustule smear for eosinophils provided data to rapidly support a clinical diagnosis of hematologic-associated EPF.     

Pustular porokeratosis

We report a case of disseminated superficial actinic porokeratosis (DSAP) with neutrophilic pustules within cornoid lamellae on histopathologic examination, corresponding to pustules along the elevated rim clinically as well. A review of the literature identified only one other case in which pustules have been associated with porokeratosis. Our case represents an unusual variant of DSAP, which may make an ordinarily straightforward clinical diagnosis more challenging.     

Paget's disease of the nipple resembling an acantholytic disease on microscopic examination

Two biopsies of an erosive lesion of the nipple had an appearance of an acantholytic disease without showing malignant cells. Only a third biopsy through the nipple with removal of a larger portion revealed some nests of atypical, large cells with clear cytoplasm, typical of Paget's disease. Immunohistochemical findings with carcinoembryonic antigen confirmed the diagnosis of Paget's disease of the nipple. This is the first case of Paget's disease which shows extensive acantholysis on microscopic examination and which resembles pemphigus vulgaris histologically. Acantholytic diseases are easily distinguished from Paget's disease and have never been mentioned in the differential diagnosis of this disease. A large biopsy through the nipple with the removal of a liberal portion of the nipple is suggested in every case of a suspected unilateral lesion of the nipple in order to avoid the overlooking of small nests of Paget's cells, as in our first biopsies, showing a histological picture of an acantholytic disease.     

Necrotizing eosinophilic folliculitis with mucinosis

A 23-year-old woman presented with a 2-week history of an extensive facial eruption consisting of markedly crusted arciform plaques covered with a haemorrhagic eschar that commenced as pruritic follicular pustules. These were associated with facial oedema but no systemic symptoms or fever. Two skin biopsies revealed prominent tissue eosinophilia with eosinophilic folliculitis and areas of follicular mucinosis, as well as haemorrhagic scale crust over the surface. The clinical and histological findings may represent an example of a necrotizing variant of eosinophilic folliculitis that has recently been reported in atopic individuals. This patient, however, did not have evidence of atopy and appeared to have a florid form of Ofuji's disease that responded rapidly to indomethacin treatment.     

Eosinophilic Pustular Folliculitis in Children after Stem Cell Transplantation: An Eruption Distinct from Graft‐Versus‐Host Disease

Eosinophilic pustular folliculitis (EPF) is a rare cutaneous disorder that typically occurs in three clinical contexts: men, individuals who are immunosuppressed or have human immunodeficiency virus, and infants. A fourth subtype occurring 2 to 3 months after hematopoietic stem cell transplantation (HSCT) has recently been described in several adults. We report two cases of EPF arising in children after HSCT. It is important to recognize this form of EPF after HSCT and differentiate it from graft‐versus‐host disease since it responds readily to topical steroids and appears to have an excellent prognosis.     

Formalin deposition as artifact in biopsies from patients affected by a new variant of endemic pemphigus foliaceus in El Bagre,Colombia, South America

Background: Most autoimmune diseases occur sporadically; however, endemic pemphigus foliaceus (EPF) is present in specific locales restricted to some geographic rural regions mostly in South America, Central America and in Tunisia (Africa). Its geographic restriction makes it an invaluable natural model for studying how the environment, genetic background and host response contribute to the development of autoimmunity. We described a new variant of EPF in El Bagre, Colombia, (El Bagre‐EPF). When we examined the skin biopsies from 10 patients and controls from the endemic area, we detected in a systematic manner several types of pigmentation, sometimes intracellular, and sometimes in the extracellular matrix in most biopsies. Aim: We aim to determine the nature of this pigment in these skin biopsies. Methods: We studied 10 patients and 10 controls matched by sex, age and work activity living in the endemic area by routine hematoxylin and eosin (H&E). Results: We were unable to find any bacteriological or parasitic organism. Specifically, we searched for several tropical disease agents as possible causative agents of this pigment. Iron stains and melanin pigment bleaching techniques failed to determine the etiology of this pigment. We then tried the removal of formalin pigment using picric acid. The pigment was removed after very strong treatment with different acids including picric acid. Conclusions: Formalin pigment shares many properties with hemozoin. In this case, the authors recommend the use of neutral buffered formalin to prevent the formation of formalin pigment especially after long periods of fixation when taking biopsies under extreme temperature and environmental humidity. Abreu Velez AM, Howard MS, Restrepo‐Isaza M, Smoller B. Formalin deposition as artifact in biopsies from patients affected by a new variant of endemic pemphigus foliaceus in El Bagre, Colombia, South America.     

Eosinophilic pustulosis: a case report

A 16-year-old female had a chronic cutaneous eruption clinically characterized by papules, pustules and large exudating plaques of 2 years duration. Multiple biopsies showed infiltration of the follicular epithelium by eosinophils and intraepidermal pustules with neutrophils and eosinophils that were accompanied either by acantholysis or spongiosis. Eosinophilia and hypoproteinemia were detected. A definite infectious etiology or autoimmune mechanism could not be established. Differential diagnosis with other entities that share some clinico-pathological and immunological features are discussed.     

Detection of mercury and other undetermined materials in skin biopsies of endemic pemphigus foliaceus

A novel variant of endemic pemphigus foliaceus (EPF) was described among individuals in an area surrounding El Bagre, Colombia, South America. The population in this rural mining community is exposed to high environmental levels of mercury, used for gold extraction, as well as other minerals, metalloids, and trace elements (e.g., quartz, rutile, granite, magnetite, and almenite) and ultraviolet radiation. Fifty control subjects and fifty EPF patients in the endemic area were examined for the presence of mercury in skin biopsies and hair, using autometallographic and mass spectroscopic analyses, respectively. Simultaneously, serum levels of IgE were measured, and cutaneous tests for hypersensitivity reactions were performed. Using autometallography, mercuric sulfides/selenides were detected in 14 of 51 skin biopsies distributed similarly in the control and patient groups. However, significantly higher serum IgE levels and mercury concentrations in hair, urine, and nails were found in patients compared with controls. Microscopic analysis revealed mercuric sulfides/selenides concentrated within and around the sweat gland epithelium, as well as in dendritic cells. Five skin biopsies from EPF patients and five from controls that tested positive for the presence of mercuric sulfides/selenides by autometallography were randomly selected for electron microscopic analysis. This analysis revealed a mixed electron-dense and electron-light material closely associated with desmosomes in patients. However, there were intracellular vesicles containing an amalgam of electron-dense and electron-light materials only in the EPF patients. Thus, EPF-affected individuals are exposed to high levels of environmental mercuric sulfides/selenides and other elements. This is the first study reporting mercuric sulfides/selenides in skin biopsies from people living in a focus of EPF, and these compounds may play a role in the pathogenesis of autoimmunity.     

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.