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

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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.

Section snippets

Methods

We performed a retrospective study of all cases of EPFI in the Hospital Niño Jesús, Madrid, Spain, between 1995 and 2011. We included only cases in which infectious processes had been ruled out and that showed tissue eosinophilia confirmed by means of smear, skin biopsy specimen, or both. We analyzed gender, age of onset, location of lesions, existence of pruritus, histologic findings, recommended treatment, duration and frequency of outbreaks, and age at resolution. We also assessed blood

Results

A total of 61 cases were evaluated, including our series of 15 cases (3 of which had previously been published in the Spanish-language literature1) and 46 more published in the literature.2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19 Twelve of the previously published cases were excluded,13, 20, 21, 22, 23, 24, 25, 26, 27 some of which had already been questioned by other authors28: 1 atypical case on both clinical and histologic grounds (genital location exclusively, dermal

Discussion

EPFI is a recurrent pruriginous condition characterized by pustular lesions on the scalp and other body areas. First described in 1984 by Lucky et al,2 the scientific literature contains many case reports and a few small series of patients,2, 3, 4, 5, 7 among which ours has gathered the largest number of cases. EPFI belongs to the group of EPF, which also includes the classic EPF or Ofuji disease, typically seen in adults, and the EPF associated with immunosuppression, mainly in

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    Funding sources: None.

    Conflicts of interest: None declared.

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