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[Eosinophilic pustulosis in an infant accompanied by immune deficit]
M Rybojad1, F Guibal, M D Vignon-Pennamen
1Service de dermatologie, Hôpital St Louis, Paris.
Insights
Juvenile eosinophilic pustulosis presents as a recurrent, itchy rash in children. Eosinophil skin infiltration suggests underlying immune dysfunction, necessitating further hematology and immunology evaluation.
Area of Science:
- Pediatric Dermatology
- Immunodermatology
- Dermatopathology
Background:
- Eosinophilic pustulosis is a rare skin condition typically presenting in early childhood.
- Characterized by itchy, papulopustular eruptions primarily on the scalp.
- Unlike in adults, the association with immune deficiency in children was previously unreported.
Observation:
- Two pediatric cases presented with recurrent, pruritic papulopustular eruptions on the scalp and trunk.
- Bacteriological and mycological investigations were negative, ruling out common infections.
- Histopathology revealed folliculitis with significant eosinophil infiltration.
Findings:
- The observed eosinophil infiltration in the skin suggests a link to immune system dysfunction.
- Both patients had a history of recurrent infections, indicating a potential underlying immunodeficiency.
- Buckley syndrome was suspected in one of the cases.
Implications:
- Juvenile eosinophilic pustulosis is part of the spectrum of childhood eosinophilic dermatoses.
- Skin eosinophil presence warrants investigation for localized or systemic immune dysfunction.
- Hematology and immunology work-up is crucial for children with associated skin or deep infections.
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.
Case Reports:
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.
Discussion:
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.