Eosinophilic pustulosis and immune deficit in childhood.

Background. Eosinophilic is a skin eruption which occurs in the first years of life, progressing by pruriginous flare-ups with amicrobial papulopustul ae on a hairless scalp. Eosinophil infiltration of the skin is variable (fo llicular or perifollicular dermal infiltration). In adults, eosinophilic pu stulosis is often associated with immune deficiency, but this association h as not been reported in children. We report two cases. Case reports. Two boys had a pruriginous papulopustular eruption involving the scalp and the trunk which had progressed with periods of exacerbation s ince birth. Search for bacteriological or mycological involvement was negat ive. Histology showed folliculitis with major polynuclear eosinophil infilt ration. Both children had a past history of repeated skin and extracutaneou s infections strongly suggesting an immune deficit. Buckly syndrome was sus pected in the second case. Discussion. Juvenile eosinophilic pustulosis belongs to the spectrum of chi ldhood eosinophilic dermatoses. The presence of eosinophil infiltration in the skin demonstrates localized or systemic immune dysfunction. A hematolog y and immunology work-up is needed in case of associated skin or deep infec tions.