P21 A rare case of eosinophilic pustular folliculitis presenting with pustules and bullae at birth

Le résumé fourni par la source

A term male neonate presented at birth with a widespread pustular dermatosis on the face, scalp and limbs, alongside bullae and erosions on the torso. Elective caesarean delivery followed an uneventful pregnancy conceived through donor-ovum in-vitro fertilisation. He was systemically well, there was sparing of the mucosal membranes, and the hair and nails were not affected. Empirical antibiotics and aciclovir were commenced and microbial skin swabs were negative. Peripheral blood eosinophilia (2.0 × 109/L) was found; and the blood film, indirect immunofluorescence, congenital infection screen and autoiummune profile were normal. Maternal history included hypothyroidism, without any other evidence of autoimmune disease nor infection. Paternal history was unremarkable, and donor history unavailable. Differential diagnoses included immunobullous disease, neonatal lupus, eosinophilic pustular folliculitis of infancy (EPFI) and transient neonatal pustular melanosis. Cutaneous histology revealed spongiosis with subcorneal eosinophil-rich pustules and non-folliculotropic inflammation. Immunostaining showed no abnormal proliferation of any cell-type. Direct immunofluorescence was negative. EPFI was diagnosed and six weeks later the lesions had spontaneously resolved. EPFI is a rare, benign condition with a median onset at 5 months, more common in males. Aetiology is speculatively due to immune dysregulation. Only one other case presenting at birth has been reported. Scalp involvement is typical. Skin biopsy shows eosinophilic infiltrates without consistent follicular involvement. Peripheral eosinophilia is frequently seen correlating with flares. Infective causes and haematological causes including Langerhans' cell histiocytosis should be excluded. EPFI often self-remits, however generalised forms, as our case, are seldom reported. Topical corticosteroids and calcineurin inhibitors are effective.

Ce résumé expose les affirmations des auteurs. BNTIC ne l’interprète pas comme une validation indépendante des résultats.