Ofuji's disease with follicular mucinosis and its differential diagnosis from alopecia mucinosa

OBJECTIVES: Ofuji's disease (OD) or eosinophilic pustular folliculitis and human immunodeficiency virus-associated eosinophilic folliculitis (HIV-EF) both show eosinophil-rich folliculocentric infiltrates, and it is not clear whether they are distinguishable pathologically. Follicular mucinosis (FM) has been observed in lesions of EF; such cases need to be differentiated from alopecia mucinosa (AM). METHODS: We compared various pathologic features in 13 cases of OD, 10 cases of non-OD-typed papular EF (seven HIV-positive and three HIV-negative), and five cases of AM. RESULTS: All cases of EF showed eosinophilic infiltrates affecting mainly the isthmus or/and sebaceous gland. Eosinophil-rich pilosebaceous pustules or/and microabscesses were noted in 69% of the biopsy specimens of OD, 73% of papular EF, 71% of HIV-EF, and 0% of AM. Mucin deposits, often abundant, were found in sebaceous lobules or/and isthmus in 41% of OD and 100% of AM. Compared with AM, OD with FM tended to show more numerous eosinophils, less abundant mucin, and most significantly, eosinophilic infundibular pustule or sebaceous microabscess. CONCLUSION: Our study suggests that OD and HIV-EF are indistinguishable pathologically and the diagnosis requires clinical correlation. FM was not uncommonly seen in lesions of OD. OD with FM may be difficult to differentiate from FM, especially in cases presenting with non-annular or non-pustular lesions, but the diagnosis might be facilitated by finding eosinophil-rich pustule, microabscess, or infiltrate in pilosebaceous units microscopically.     

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.     

Papular follicular eruptions in human immunodeficiency virus-positive patients in South Africa

BACKGROUND: Papular and follicular eruptions, such as papulopruritic eruption, eosinophilic folliculitis, and infective folliculitis, are relatively common disorders in patients infected with the human immunodeficiency virus (HIV). These conditions may show considerable clinical overlap. OBJECTIVE: To assess the relative proportion of pruritic papular cutaneous eruptions in South Africans with HIV-associated dermatoses, and to correlate the clinical and histologic features of these lesions. METHODS: The clinical and histologic features of papular follicular eruptions were correlated in 40 consecutive black HIV-positive patients who underwent skin biopsy. RESULTS: The clinical features were similar in all patients and consisted of widespread papules and pustules involving the face, limbs, and trunk. The most common histologic finding was acute suppurative folliculitis, seen in 27 patients (67.5%). In most cases, no cause was found for the suppuration. Papulopruritic eruption of HIV was diagnosed in six patients (15%), HIV-associated eosinophilic folliculitis in four (10%), Pityrosporum folliculitis in two (5%), and acne in one (2.5%). Concordance between the initial clinical diagnosis and the final histopathologic diagnosis was achieved in only 27.5% of cases. CONCLUSION: Skin biopsy remains an important adjunct to the correct diagnosis and classification of papular and follicular eruptions in HIV-positive patients.     

Eosinophilic folliculitis in 2 HIV-positive women

BACKGROUND: Human immunodeficiency virus-associated eosinophilic folliculitis (HIV-EF) among homosexual men is a commonly reported dermatologic finding, while only 4 cases in HIV-positive women have been documented in the literature to date. This article describes 2 additional cases of HIV-EF in immunocompromised women and reviews the data on this condition. OBSERVATIONS: The diagnoses were made on the basis of clinical appearance and microscopic analysis of skin biopsies. The women were not receiving highly active antiretroviral therapy (HAART) and their CD4 cell counts were below 100/ micro L. CONCLUSIONS: As HIV prevalence continues to increase in the female population, more cases of HIV-EF will be seen among women. Because the etiology of HIV-EF remains elusive, no single treatment stands above the rest although several successful therapies have been demonstrated. However, HAART restores the proper T-cell milieu, which seems to improve the course of this disease.     

Ofuji's disease: a report on 20 patients with clinical and histopathologic analysis

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. OBJECTIVES 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.     

Tinea infection histologically simulating eosinophilic pustular folliculitis

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.     

Eosinophilic pustular folliculitis

Eosinophilic pustular folliculitis is a rase dermatosis and the treatment is difficult because the underlying pathogenic mechanism is unknown. The authors report a case of eosinophilic pustular folliculitis (Ofuji's disease) in a 50-year-old man who died 9 years after onset of the disease.     

Human immunodeficiency virus-associated eosinophilic folliculitis. A unique dermatosis associated with advanced human immunodeficiency virus infection.

We studied 13 patients with human immunodeficiency virus (HIV) infection and a chronic pruritic folliculitis that was unresponsive to systemic treatment with bactericidal anti-staphylococcal antibiotics. The skin eruption was characterized by multiple urticarial follicular papules scattered on the trunk (100%), the head and neck (85%), and the proximal aspect of the extremities (62%). Absolute peripheral eosinophil counts were increased in six of 13 patients; a relative peripheral eosinophilia was present in 10 of 13 patients. Serum IgE levels were elevated in all seven patients tested (range, 88 to 9050 IU). Histopathologic features included a folliculitis with eosinophils. Pathogenic bacteria were not consistently found by routine bacterial skin cultures, cultures of skin biopsy specimens, or histopathologic evaluation. CD4 counts were decreased in all of the 12 patients tested (less than 300 cells per cubic millimeter) and were below 250 cells per cubic millimeter in 10 patients. A clinical response was noted to astemizole, to ultraviolet light in the B range, and to topical clobetasol propionate. These observations demonstrate that HIV-associated eosinophilic folliculitis is a unique HIV-related cutaneous disorder that is characterized by a culture-negative, chronic, pruritic folliculitis and a characteristic histopathologic picture. Of special importance, because it is associated with CD4 counts of less than 250 to 300 cells per cubic millimeter, eosinophilic folliculitis appears to be an important clinical marker of HIV infection and, particularly, of patients at increased risk of developing opportunistic infections. We suggest that the term eosinophilic pustular folliculitis (Ofuji's disease), previously used to describe this dermatosis in HIV-infected patients, should be discarded.     

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.     

HIV-associated eosinophilic folliculitis: case report and review of the literature

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.     

Eosinophilic folliculitis in a Caucasian patient: association with toxocariasis?

Sterile eosinophilic folliculitis, a clinical entity first described by Ofuji in 1970, is a rather rare skin disorder, in particular in the non-Asian population. We report the first case of eosinophilic folliculitis associated with toxocariasis in a Caucasian patient. Topical and systemic anti-inflammatory and antiphlogistic therapy along with systemic antihelminthic treatment resulted in complete remission of the skin lesions. In addition, there was a marked decrease of antibodies to Toxocara antigens in the patient's serum following antihelminthic therapy. Given that (I) some cases of eosinophilic folliculitis have been reported which were associated with infestation with metazoan parasites; (2) infestations with the roundworm Toxocara canis are known to induce eosinophilic reactions in some tissues; and (3) therapy-induced remission of eosinophilic folliculitis was accompanied by a decrease of Toxocara-directed antibodies in the patient's serum, we propose that there is an aetiopathogenic link between toxocariasis and eosinophilic folliculitis in this patient.     

Eosinophilic Folliculitis in HIV-Infected Women

Background and objective: Dermatologic conditions are often presenting signs of HIV infection and may be the sole cause of morbidity in patients who have otherwise stable HIV disease. Eosinophilic folliculitis is a pruritic, follicular eruption that typically manifests late in the course of HIV infection. Most published reports of eosinophilic folliculitis have been in HIV-infected men. In those reports, a characteristic truncal distribution was present, with involvement of the head, neck, and upper extremities commonly seen as well. The objective of this study was to better characterize the presentation of eosinophilic folliculitis in women. Methods: We conducted a retrospective chart review of six HIV-seropositive women with eosinophilic folliculitis previously seen in our dermatology clinics. We also reviewed the literature for cases of eosinophilic folliculitis in women and for clinical and therapeutic aspects of the condition, particularly in women. Results: In our case series, we found that eosinophilic folliculitis in women may predominantly affect the face and mimic acne excoriée. A review of the literature of HIV-associated eosinophilic folliculitis in women supports these findings. Regarding treatment, many therapies are available, but none is uniformly effective. Conclusion: Given the dramatic rise in the incidence of HIV infection in women, who now represent nearly 50% of adults living worldwide with HIV/AIDS, a heightened awareness of HIV-related dermatoses in women is essential. HIV-associated eosinophilic folliculitis should be considered in the differential diagnosis of chronic, pruritic, papular facial eruptions in females.     

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 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.     

HIV-Associated Eosinophilic Pustular Folliculitis: Successful Treatment of a Japanese Patient with UVB Phototherapy

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).     

Eosinophilic pustulosis of the scalp in childhood.

BACKGROUND: Among sterile pustulosis 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.     

Pruritic eosinophilic papular eruption revealing HIV infection

Eosinophilic folliculitis (EF) is a rare follicular pruritic papular eruption observed in association with human immunodeficiency virus (HIV). The diagnosis of eosinophilic folliculitis is based on the histologic findings consisting of a sterile inflammatory infiltrate rich in eosinophils involving hair follicles. EF in HIV patients is believed to be an immunoinflammatory response directed either at follicular or skin flora antigens in the late-stage of HIV infection. In this stage, immune response is characterized by a shift from a Th1- to a Th2-dominant cytokine profile and an increased secretion of interleukin-4 and interleukin-5, both known to promote eosinophilia. We describe a case of HIV-associated eosinophilic folliculitis in a 30-year-old black woman referred to us for a pruritic follicular eruption without any other clinical symptom related to the acquired immunodeficiency syndrome. HIV infection presenting with EF has been rarely reported and its occurrence in women is also very rare.     

Eosinophilic pustular folliculitis (Ofuji's disease) with response to dapsone therapy

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.     

Itchy folliculitis and human immunodeficiency virus infection: clinicopathological and immunological features, pathogenesis and treatment

The predominant itchy folliculitis associated with human immunodeficiency virus (HIV) infection appears to be an eosinophilic folliculitis (EF). This is characterized by lytic degeneration of sebaceous glands and an inflammatory infiltrate in which eosinophils and CD8+ T lymphocytes predominate. All patients have low CD4 counts and present late on in their HIV disease. Lesional distribution is mainly truncal, with a significant proportion also having facial involvement. Our prospective survey has shown that it is impossible to differentiate clinically between infective folliculitis and EF, and we recommend therefore that all cases are biopsied. We review the clinicopathological and immunological aspects of HIV-associated itchy folliculitis, in particular HIV-associated EF as well as current theories on pathogenesis and treatment. We suggest that HIV-associated EF is an autoimmune disease with the sebocyte or some constituent of sebum acting as the autoantigen.