Eosinophilic pustular folliculitis of infancy: a series of 15 cases and review of the literature.

BACKGROUND: Eosinophilic pustular folliculitis (EPF) of infancy is characterized by the presence of pustular lesions containing eosinophils. It is the least well-characterized of the EPF diseases. OBJECTIVES: We sought to define the clinical and histopathologic features of the condition. METHODS: We conducted a retrospective review of the clinical data and histologic findings of 15 patients given the diagnosis of EPF of infancy at the Hospital Niño Jesús, Madrid, Spain, from 1995 to 2011, and of patient data published in MEDLINE with such a diagnosis from the disease description (1984-2011). RESULTS: A total of 61 cases were collected. The disease was more common in males than females (ratio 4:1), and presented before 14 months of life in 95% of cases (mean 6.1 months; median 5 months). All patients had recurrent outbreaks and scalp involvement, and 65% had lesions on areas of the body other than the scalp. Tissue eosinophilia was present in all cases; however, true follicular involvement was observed only in 62% of cases in which histologic study was available. More than 80% of the patients were cured by 3 years of age (mean 25.3 months; median 18 months). Topical steroids were effective in 90% of cases. LIMITATIONS: This was a retrospective study. CONCLUSIONS: EPF of infancy presents most often in the first 14 months of life and usually resolves by 3 years of age. All patients showed scalp involvement, tissue eosinophilia, and recurrent outbreaks. The condition does not require aggressive treatment, as it is benign and self-limiting.

Analysis of serum chemokine levels in patients with HIV-associated eosinophilic folliculitis.

BACKGROUND: Patients with human immunodeficiency virus (HIV) infection exhibit various skin diseases. HIV-associated eosinophilic folliculitis (EF) and pruritic papular eruption (PPE) are frequently seen. OBJECTIVE: To understand the mechanisms underlying HIV-associated EF and PPE. METHODS: In order to know frequencies of EF and PPE among patients with HIV infection, we first collected HIV(+) patients who visited dermatology clinic in National Center for Global Health and Medicine during February 2007. We next collected 25 serum samples from HIV(+) patients with skin diseases from May 2008 to May 2010. Eight of 25 patients had EF (EF group), four had PPE (PPE group) and others had non-itchy skin problems such as condyloma acuminatum (no itch group). RESULTS: We first confirmed high frequencies of EF (10.7%) and PPE (5.3%) among 75 HIV(+) patients who visited our clinic during one month. We then measured serum levels of CCL11, CCL17, CCL26 and CCL27. Serum CCL17 levels in EF were significantly higher than those of PPE and no itch group. Serum CCL26 and CCL27 levels in EF were higher than those of no itch group. The number of CD4(+) cells in EF was significantly lower than that in no itch group. CONCLUSION: High serum levels of CCL17, CCL26 and CCL27, and low CD4(+) cell counts may account for the development of HIV-associated EF.

Neutrophil folliculitis and serum androgen levels.

Pruritic folliculitis (PF) is one of the specific dermatoses of pregnancy the aetiology of which remains unknown. Several theories have been proposed, and the most commonly accepted is that it represents a hormonally induced acne, as seen in patients taking progestogenic steroids. This theory led to the proposition that serum androgen levels may be elevated in this condition, and may be causative. We studied a cohort of 125 pregnant women presenting with a variety of pregnancy dermatoses of whom 12 had PF. These women were compared with 138 pregnant control subjects without a pregnancy dermatosis to determine whether serum androgens were elevated. There was no significant elevation of serum androgens in the 12 cases of PF. There was an overall preponderance of male infants among the PF cases and birthweight of the infants was found to be reduced compared with those of controls. However, the outcome of pregnancy in all cases was favourable implying that PF has no adverse effect on foetal wellbeing.

A prospective study of 200 women with dermatoses of pregnancy correlating clinical findings with hormonal and immunopathological profiles.

In 1994 we set up a specialist clinic for pregnancy dermatoses, both to improve the management of pregnant women with skin problems and to enhance our general understanding of the pregnancy dermatoses. This clinic has provided a large database of 200 women which has formed the basis for a prospective study over a 2-year period. In each case the dermatological diagnosis was clearly defined on clinical criteria, with additional help from histopathology and direct immunofluorescence of the skin where appropriate. We have included a number of patients who presented with relatively trivial diagnoses, as this reflects the referral patterns of our midwives, general practitioners and obstetricians within our hospital and local population. Our results show that all patients with specific dermatoses of pregnancy conformed well to the classification established by Holmes and Black in 1983. The role of the sex hormones [oestradiol, human chorionic gonadotrophin (hCG) and cortisol] in polymorphic eruption (PEP) and prurigo of pregnancy was studied in 125 cases and compared with 138 normal healthy pregnant controls. For pruritic folliculitis (PF), serum androgens were measured to establish if these were elevated. Nearly all patients were followed up postpartum, with respect to both maternal and fetal prognosis (some were unfortunately lost to follow-up). Many patients were primiparous (47%) and presented in their third trimester (49%). This study shows a surprisingly high prevalence of eczema during pregnancy. It is possible that earlier cases in the literature termed prurigo of pregnancy may in fact have been eczema, thus explaining the low incidence of prurigo in this study. Hormonal analysis showed a significant reduction in serum cortisol levels in patients with PEP compared with normal pregnant controls (P = 0.03), although hCG and oestradiol showed no differences. Serum androgens were not significantly elevated in patients with PF compared with controls. Birthweight (analysed by the individualized birthweight ratio) was significantly reduced in both the PF and pemphigoid gestationis groups. In the PEP and PF groups there was a male/female infant ratio of 2 : 1, not noted in previous studies. In all cases studied there were no adverse effects either on maternal or fetal outcome as a result of the pregnancy dermatosis. This study indicates that all patients fulfilled the criteria of the previous classification of the specific dermatoses of pregnancy, although we also now highlight the frequency of eczema in pregnancy and speculate as to possible causes. There were no cases of papular dermatitis of pregnancy. We feel that the specialist clinic is an important service which has improved the management of these women and identified areas for further research.

Ofuji's disease and cytokines: Remission of eosinophilic pustular folliculitis associated with increased serum concentrations of interferon gamma.

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.

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) (author's transl)]. / Folliculite à éosinophiles (maladie d'Ofuji).

A case, the first French observation of eosinophilic pustular folliculitis (Ofuji) is reported. The long lasting condition of unknown etiology is characterized by slightly indurated erythematous plaques and formation of follicular small papules and pustules. The lesions have a tendency to central healing and peripheral extension and proceed with remissions and exacerbations. Histological features are pustular folliculitis composed chiefly of eosinophiles. Blood eosinophila is often founded. Local corticotherapy and dapsone are sometimes a effective treatment.

[Sterile eosinophilic pustulosis (author's transl)]. / Sterile eosinophile Pustulose.

'Eosinophilic pustular folliculitis", first described by Ofuji and co-workers in 1970, is obviously a new entity, not only confined to Japan. Recently, there was a case reported from Sweden; we present here the first observation in the FRG. The disease is characterized by eruptions of aggregated pustules, containing numerous eosinophils, and by blood eosinophilia. In our patient the disease first appeared on the scalp, leading to scarring alopecia. Since in our and in some other patients the lesions were not limited to the follicles, we feel that the term 'sterile eosinophilic pustulosis" is more appropriate to reflect the clinical picture. For differential diagnosis Morbus Duhring and M. Sneddon-Wilkinson have to be considered. "Sterile eosinophilic pustulosis" differs from these mainly because of the eosinophilic infiltrations deep in the dermis. Immunofluorescence tests are negative. Treatment with DADPS may be helpful.