Eosinophilic pustular folliculitis in infancy: report of two affected brothers

Abstract

Summary We describe two brothers with eosinophilic pustular folliculitis. Both had recurrent crops of papules and pustules, primarily affecting the scalp. The eruption began in the neonatal period. Both children had a leucocytosis with eosinophilia. Histology revealed folliculitis, with an infiltrate in which eosinophils were predominant. Bacteriological and fungal cultures of pustules were negative. There was a good clinical response to treatment with a topical corticosteroid and dapsone. We review the 28 previously reported cases of eosinophilic pustular folliculitis in infancy. The occurrence of this disorder in brothers suggests that an inherited or contagious factor may be involved in its aetiology.

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

“…Regarding treatment, 51 of the 61 children were treated with low-to medium-potency topical steroids (83%), of whom 46 responded well, meaning that they were effective in 90% of cases in which their use was specified. Of the 5 nonresponders, 1 responded well to oral erythromycin, 3 2 required dapsone, 5,9 and in 2 additional cases the alternative treatment was not detailed. 2,14 Dapsone was effective in both cases, but had to be withdrawn in 1 case because of hematologic side effects.…”

“…These were isolated cases, 3,5,7 whereas many patients were treated ineffectively with antibiotics before being given the diagnosis. [2][3][4][5][6][7][8][9]11,12,14,15,17,19 The good response could be considered as coincidental, because the process tends to be self-limiting, even without treatment, 15,18 but some authors favor the antichemotactic activity of some antibiotics such as erythromycin. 7 Cetirizine has also been successfully used by some authors, who advocate its antieosinophilic activity and antipruritic effect, 3 as has cimetidine.…”

“…[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, 13,20-27 some of which had already been questioned by other authors 28 : 1 atypical case on both clinical and histologic grounds (genital location exclusively, dermal infiltrate rich in neutrophils in 1 biopsy specimen, and in lymphocytes and macrophages in another) 25 ; 3 cases associated with immunodeficiency 26,27 ; 1 patient who developed sepsis with hepatic and pulmonary involvement in whom immunodeficiency was not ruled out 13 ; and 1 exceptional case in which the eruption did not recur, the histologic findings were inconclusive, and that was treated with granulocyte-colony stimulating factor (G-CSF) because of the extraordinarily high eosinophilia. 22 There were 6 more cases that were also excluded because of their atypical manifestations: a 4-year-old child who had recurrent vesicles and pustules along with erythema multiformeelike lesions on the abdomen and lower limbs that might have corresponded to arthropod bites 23 ; an 8-yearold boy with mucosal involvement, mixed dermal inflammatory infiltrate with eosinophils and neutrophils, and good response to dapsone in whom linear IgA was not excluded 20 ; an 11-year-old girl who presented with an isolated annular plaque on the cheek showing a dense eosinophilic infiltrate and necrotizing vasculitis of the small vessels, 24 which is not a feature of EPFI; a 5-year-old girl with ulcerated lesions on the lower legs that resolved in 2 weeks 21 ; and 2 more patients who presented with crops of pustular lesions on the trunk and lower limbs for 3 months' duration who demonstrated hypersensitivity to Dermatophagoides pteronyssinus and elevated IgE levels.…”

Diseases of cutaneous appendages

“…243 More recently it has been suggested that the production of nitric oxide by eosinophils may have a pathogenetic role. [261][262][263][264] Onset can be as early as the first day of life. 231,[245][246][247][248][249][250][251][252][253] It differs from the classic form (Ofuji's disease) by the severe pruritus, the absence of circinate and palmoplantar lesions, and the less frequent involvement of the face.…”

HIV seronegative eosinophilic pustular folliculitis successfully treated with doxicycline

“…Several authors reported cases of this disease in immunocompromised or oncohaematological caucasian patients, either human immunodeficiency virus (HIV)‐positive or treated with immunosuppressory agents 2,3 . A childhood variant was also identified, characterized by the perinatal occurrence of lesions 4,5 . EPF has been rarely described in association with drug as carbamazepine 6 or allopurinol 7 …”