[Eosinophilic pustulosis in an infant accompanied by immune deficit].
BACKGROUND
Eosinophilic is a skin eruption which occurs in the first years of life, progressing by pruriginous flare-ups with amicrobial papulopustulae on a hairless scalp. Eosinophil infiltration of the skin is variable (follicular or perifollicular dermal infiltration). In adults, eosinophilic pustulosis is often associated with immune deficiency, but this association has not been reported in children. We report two cases.


METHODS
Two boys had a pruriginous papulopustular eruption involving the scalp and the trunk which had progressed with periods of exacerbation since birth. Search for bacteriological or mycological involvement was negative. Histology showed folliculitis with major polynuclear eosinophil infiltration. Both children had a past history of repeated skin and extracutaneous infections strongly suggesting an immune deficit. Buckly syndrome was suspected in the second case.


CONCLUSIONS
Juvenile eosinophilic pustulosis belongs to the spectrum of childhood eosinophilic dermatoses. The presence of eosinophil infiltration in the skin demonstrates localized or systemic immune dysfunction. A hematology and immunology work-up is needed in case of associated skin or deep infections.
Rybojad
M
M
Service de dermatologie, Hôpital St Louis, Paris.
Guibal
F
F
Vignon-Pennamen
M D
MD
Prigent
F
F
Morel
P
P
Bourrat
E
E
D000328:Adult; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D004802:Eosinophilia / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D004804:Eosinophils / Q000473:pathology; D005499:Folliculitis / Q000175:diagnosis / Q000276:immunology / Q000473:pathology; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D008297:Male; D012535:Scalp / Q000473:pathology; D012867:Skin / Q000473:pathology; D012872:Skin Diseases, Vesiculobullous / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D013577:Syndrome