For many years, this was a rare skin rash, predominately found on the head and neck of Asian patients. However, with the arrival of AIDS, patients began developing lesions which histologically and clinically resembled this rare disease. With time, these cases became known as HIV associated eosinophilic folliculitis.
Epidemiology Disease Associations Pathogenesis Laboratory/Radiologic/Other Diagnostic Testing Gross Appearance and Clinical Variants Histopathological Features and Variants Special Stains/Immunohistochemistry/Electron Microscopy Differential Diagnosis Prognosis and Treatment Commonly Used Terms
EPIDEMIOLOGY CHARACTERIZATION SYNONYMS Eosinophilic folliculitis of Ofuji GEOGRAPHY
Eosinophilic pustular folliculitis.
Jaliman HD, Phelps RG, Fleischmajer R.
J Am Acad Dermatol 1986 Mar;14(3):479-82 Abstract quote
We present the case of an American adult patient with eosinophilic pustular folliculitis. The majority of previously reported patients have been Japanese. Eosinophilic pustular folliculitis is a rare condition characterized by recurrent, peripherally expanding, sterile papulopustules and plaques. Hair-bearing areas such as the face, chest, back, and extensor surfaces of the upper arms are usually involved, although palms and soles can be affected as well.
Histologic examination shows subcorneal and intrafollicular abscesses and spongiosis of the outer root sheath. The eosinophil is a prominent infiltrating cell, although mononuclear cells and neutrophils may also be seen. There is often a dense perivascular infiltrate of eosinophils in the dermis. Our patient was unusual in that he did not respond to multiple therapeutic regimens.
Eosinophilic pustular folliculitis. Report of a European case.
Br J Dermatol 1976 Dec;95(6):661-4 Abstract quote
A case of eosinophilic pustular folliculitis (Ofuji) is presented. This long-lasting disease of unknown aetiology has hitherto been reported only from Japan. It is characterized by tinea-like slowly growing rings and by blood eosinophilia.
The histological changes are epidermal infiltrations and abscesses of predominantly eosinophilic leukocytes, often localized to the follicles.
DISEASE ASSOCIATIONS CHARACTERIZATION DRUG ALLERGY
A case of eosinophilic pustular folliculitis (Ofuji's disease) induced by patch and challenge tests with indeloxazine hydrochloride.
Kimura K, Ezoe K, Yokozeki H, Katayama I, Nishioka K.
Department of Dermatology, Tokyo Medical and Dental University, Japan
J Dermatol 1996 Jul;23(7):479-83 Abstract quote
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 in patients with acquired immunodeficiency syndrome. Report of three cases.
Soeprono FF, Schinella RA.
J Am Acad Dermatol 1986 Jun;14(6):1020-2 Abstract quote
Eosinophilic pustular folliculitis is a rare disease. Highly unusual is its occurrence in three patients with acquired immunodeficiency syndrome (AIDS), described herein. This association may be the result of immunologic aberrations that are described in AIDS and may represent an abnormal reaction to skin saprophytes and dermatophytes.
Eosinophilic pustular folliculitis (Ofuji's disease) and non-Hodgkin lymphoma.
Patrizi A, Di Lernia V, Neri I, Gherlinzoni F.
Department of Dermatology, L. e A. Seragnoli, University of Bologna, Italy.
Acta Derm Venereol 1992;72(2):146-7 Abstract quote
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.
Eosinophilic pustular folliculitis (Ofuji's disease) in myelodysplastic syndrome.
Jang KA, Chung ST, Choi JH, Sung KJ, Moon KC, Koh JK.
Department of Dermatology, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea.
J Dermatol 1998 Nov;25(11):742-6 Abstract quote
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.
PATHOGENESIS CHARACTERIZATION CYTOKINES
Ofuji's disease and cytokines: Remission of eosinophilic pustular folliculitis associated with increased serum concentrations of interferon gamma.
Teraki Y, Imanishi K, Shiohara T.
Department of Dermatology, Kyorin University School of Medicine, Japan.
Dermatology 1996;192(1):16-8 Abstract quote
Background and Objective: Eosinophilic pustular folliculitis (EPF) is a distinct inflammatory dermatosis of unknown etiology. The aim of this study is to evaluate the serum levels of cytokines in 3 patients with EPF before and after treatment with indomethacin.
Results: Serum levels of interleukin 4 (IL-4) were elevated in patients with EPF. The remission of EPF was associated with increased serum concentrations of interferon gamma with no essential change of serum levels of IL-4.
Conclusion: These results suggest that subtle changes in serum levels of cytokines are involved in the remission of EPF and that indomethacin may provide the remission of EPF by altering cytokine production in patients with EPF.
Evidence of retroviral involvement in an Italian patient with Ofuji's disease.
Veraldi S, Ferrante P, Mancuso R, Gianotti R, Caputo R.
Institute of Dermatological Sciences, IRCCS, University of Milan, Milan, Italy.
Dermatology 1999;198(1):86-9 Abstract quote
Ofuji's disease, also known as 'eosinophilic pustular folliculitis', is a rare inflammatory skin disorder of unknown aetiology, which affects mostly adult Japanese males. Only few Caucasian patients have been described so far.
We report the case of a 44-year-old Italian woman with Ofuji's disease who had circulating antibodies against the recombinant envelope glycoprotein (Rgp46I) of HTLV-1. By means of a very sensitive nested PCR, the tax region of HTLV-1, but not other regions, was found in peripheral blood mononuclear cells. Our observations suggest that a retrovirus, closely related to HTLV but not yet identified, could be involved in the pathogenesis of the disease in this patient.
CHARACTERIZATION RADIOLOGIC LABORATORY MARKERS
Ofuji's disease: high circulating titers of IgG and IgM directed to basal cell cytoplasm.
Nunzi E, Parodi A, Rebora A.
J Am Acad Dermatol 1985 Feb;12(2 Pt 1):268-73 Abstract quote
A patient with Ofuji's eosinophilic pustulosis and peculiar immunofluorescence findings is described.
High titers of circulating IgG and IgM have been found directed to the cytoplasm of the basal cells of epidermis and the outer sheath of hair follicles. Such antibodies appear to be related to the course of the disease.
Eosinophilic pustular folliculitis.
Colton AS, Schachner L, Kowalczyk AP.
J Am Acad Dermatol 1986 Mar;14(3):469-74 Abstract quote
Eosinophilic pustular folliculitis was first described by Ofuji et al in 1970. It is characterized by pruritic circinate plaques that are studded with follicular papules and pustules. Lesions are located chiefly on the face, trunk, and arms.
Biopsies of lesions demonstrate an infiltrate of eosinophils and neutrophils within hair follicles, dermis, and epidermis. Peripheral leukocytosis and eosinophilia are common.
Ofuji's disease: A report on 20 patients with clinical and histopathologic analysis.
Ishiguro N, Shishido E, Okamoto R, Igarashi Y, Yamada M, Kawashima M.
Department of Dermatology, Tokyo Women's Medical University.
J Am Acad Dermatol 2002 Jun;46(6):827-33 Abstract quote
BACKGROUND: Ofuji's disease, also referred to as classic eosinophilic pustular folliculitis, is a rare dermatosis of unknown origin for which there is no uniformly effective treatment.
Objective and Methods: Twenty patients with Ofuji's disease (classic eosinophilic pustular folliculitis) evaluated in our department from January 1978 to June 1999 were studied clinically and histopathologically. Laboratory data, treatments, and clinical courses were evaluated in 12 of these individuals.
RESULTS: Nissl modified staining revealed moderate increases of mast cells around hair follicles and sebaceous glands in all 20 patients; the majority of the infiltrating mast cells were tryptase-positive and chymase-negative. Eight of 11 patients treated with oral indomethacin responded completely.
CONCLUSIONS: Indomethacin is an effective therapy for Ofuji's disease. Tryptase-positive and chymase-negative mast cells might play some role in the pathogenesis of Ofuji's disease.
Eosinophilic pustular folliculitis in infancy.
Lucky AW, Esterly NB, Heskel N, Krafchik BR, Solomon LM.
Pediatr Dermatol 1984 Jan;1(3):202-6 Abstract quote
Five infants under 1 year of age were reported with a syndrome of recurrent crops of pruritic papulopustules of the scalp. In three children there were also intermittent outbreaks on the trunk and extremities. Cultures showed the pustules to be sterile. Biopsies of scalp and skin tissues showed eosinophilic folliculitis. Some patients had eosinophilia during outbreaks of pustules.
These cases are similar to the eosinophilic pustular folliculitis reported in a few adult patients with the exception that there was predominant scalp involvement in the children. We propose that eosinophilic pustular folliculitis of infancy is a distinct pustular dermatosis.
Eosinophilic pustular folliculitis (Ofuji disease) in childhood: a review of four cases.
Giard F, Marcoux D, McCuaig C, Powell J, Russo P.
Department of Pediatrics, Universite de Montreal, Ste-Justine Hospital, Quebec, Canada.
Pediatr Dermatol 1991 Sep;8(3):189-93 Abstract quote
Four children under 2 years of age were treated at our hospital in the last three years with a recurrent vesiculopustular eruption of the scalp, variably extending to the face and limbs. All cases followed a cyclical pattern. Three of the children had a moderate response to topical steroids, and one went into remission after a two-month course of erythromycin. Ethnic origin may be an important predisposing factor.
Histologically, all patients showed a moderate mixed inflammatory infiltrate with numerous eosinophils centered around hair follicles. Peripheral white blood cell count showing leukocytosis with eosinophilia was observed in those cases measured, but no consistent immunologic abnormalities could be identified.
HIV-associated eosinophilic folliculitis in an infant.
Ramdial PK, Morar N, Dlova NC, Aboobaker J.
Department of Pathology, Faculty of Medicine, University of Natal, South Africa.
Am J Dermatopathol 1999 Jun;21(3):241-6 Abstract quote
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.
HISTOLOGICAL TYPES CHARACTERIZATION GENERAL Hair follicles expanded by a populations of eosinophils and neutrophils VARIANTS
CHARACTERIZATION SPECIAL STAINS IMMUNOPEROXIDASE GENERAL
Eosinophilic pustular folliculitis (Ofuji's disease). Immunohistochemical analysis.
Teraki Y, Konohana I, Shiohara T, Nagashima M, Nishikawa T.
Department of Dermatology, Hiratsuka City Hospital, Kanagawa, Japan.
Arch Dermatol 1993 Aug;129(8):1015-9 Abstract quote
BACKGROUND: Eosinophilic pustular folliculitis is a distinctive dermatosis that was first described in Japan. Although the histopathologic feature of eosinophilic pustular folliculitis is characterized by follicular infiltrates with numerous eosinophils, its pathophysiology remains unclear. The lesional skin of five patients with eosinophilic pustular folliculitis was examined using several monoclonal antibodies including a variety of anti-leukocyte adhesion molecules: endothelial cell adhesion molecule 1, vascular cell adhesion molecule 1, and intercellular adhesion molecule 1, by means of immunohistochemical techniques.
OBSERVATION: Intercellular adhesion molecule 1 expression by keratinocytes was observed on follicular epithelium, but not on epidermis. The migration of eosinophils and lymphocytes, which were intensely positive for antilymphocyte function-associated antigen 1, was limited to follicular epithelium. Endothelial cell adhesion molecule 1 and vascular cell adhesion molecule 1 expression of vascular endothelium was more often observed around hair follicles. There was no reactivity for interleukin 8 in follicular epithelium.
CONCLUSIONS: These findings may explain the selective migration of eosinophils and lymphocytes to the hair follicles in eosinophilic pustular folliculitis.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES TINEA FACIALE
Tinea infection histologically simulating eosinophilic pustular folliculitis.
Kuo TT, Chen SY, Chan HL.
A biopsy taken from an annular eruption on the thigh of an infant boy show
J Cutan Pathol 1986 Apr;13(2):118-22 Abstract quote
A biopsy taken from an annular eruption on the thigh of an infant boy showed features of Ofuji's eosinophilic pustular folliculitis, yet fungal hyphae were demonstrated in the lesion.
This case is reported to illustrate that tinea infection can stimulate eosinophilic pustular folliculitis histologically.
PROGNOSIS AND TREATMENT CHARACTERIZATION PROGNOSTIC FACTORS TREATMENT GENERAL
Ofuji's disease: diagnostic and therapeutic problems. A report of three cases.
Patrone P, Bragadin G, Stinco G, De Francesco V, Frattasio A.
Institute of Dermatology, DPMSC, University of Udine, Italy.
Int J Dermatol 2001 Aug;40(8):512-5 Abstract quote
BACKGROUND: Ofuji's disease is an eosinophilic dermatosis affecting mostly male subjects. It is characterized by the appearance of follicular papulopustules, at times on an erytemathous base, which tend to form an annular configuration. The evolution is relapsing remitting. The histopathological examination demonstrates a dense dermal infiltrate with a prevalence of perifollicular and perivascular eosinophilia. Peripheral blood eosinophilia is observed in a high percentage of cases. The pathogenesis is unclear.
METHODS: We report three cases of patients affected by Ofuji's disease with presentation on different sites. Our case reports concern three male subjects otherwise in good health and whose hematologic results were normal. Before they came to our observation, an erroneous diagnosis had been made and they had been subjected to improper treatment.
RESULTS: To make a definite diagnosis it was necessary to evaluate the following features as a whole: the clinical aspect, the evolution, the result of the histological examination, the negativity of cutaneous cultures and the lack of response to previous treatments. All three patients were treated with dapsone 100 mg/day with regression of the clinical manifestations. In one case there was a relapse which was treated with isotretinoin 0.5 mg/kg/day.
CONCLUSIONS: From our experience we can deduce that in cases of follicular pathologies which do not respond to conventional therapies, it is necessary to take into consideration the possibility of Ofuji's disease and, in that case, patients should be monitored because of the frequent relapses.
Eosinophilic pustular folliculitis (Ofuji's disease) with response to dapsone therapy.
Arch Dermatol 1985 Jul;121(7):921-3 Abstract quote
A 50-year-old man had eosinophilic pustular folliculitis (Ofuji's disease) characterized by follicular pustular papules on the face, confluent vesicles on the fingers, and a papulopustular area on the upper portion of the back. Extensive examinations and cultures of both pustular material and tissue revealed no organisms, except Staphylococcus epidermidis.
The disease responded to dapsone therapy.
Eosinophilic pustular folliculitis (Ofuji's disease): efficacy of isotretinoin.
Berbis P, Jancovici E, Lebreuil G, Benderitter T, Dubertret L, Privat Y.
Clinique Dermatologique, Hotel Dieu, Marseille, France.
Dermatologica 1989;179(4):214-6 Abstract quote
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 (Ofuji's disease): indomethacin as a first choice of treatment.
Ota T, Hata Y, Tanikawa A, Amagai M, Tanaka M, Nishikawa T.
Department of Dermatology, Keio University School of Medicine, Tokyo, Japan.
Clin Exp Dermatol 2001 Mar;26(2):179-81 Abstract quote
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: successful treatment with interferon-alpha.
Mohr C, Schutte B, Hildebrand A, Luger TA, Kolde G.
Department of Dermatology, Westfalische Wilhelms-Universitat, Munster, Germany.
Dermatology 1995;191(3):257-9 Abstract quote
Eosinophilic pustular folliculitis (Ofuji's disease) is a rare skin disease of unknown etiology characterized by infiltrated circinate plaques with sterile follicular pustules in primarily seborrheic areas.
Several therapeutic regimens have been reported to control the disease with inconsistent results. We here report on a patient with Ofuji's disease, who was successfully treated with interferon-alpha 2b.
Weedon D. Weedon's Skin Pathology. Churchill Livingstone. 1997.
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
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