Abstract
We herein present a 4 year-old boy with Waardenburg-Shah syndrome who developed Jacquet erosive diaper dermatitis following a total colectomy and ileoanal anastomosis procedure for Hirschsprung disease. The diagnosis was made according to history and typical clinical findings. Complete resolution of the recalcitrant lesions after an ileostomy procedure supported the diagnosis. This case highlights the importance of being familiar with the predisposing factors and clinical presentation of this rare and severe form of chronic irritant dermatitis, since it may easily be misdiagnosed as other diseases in children and may lead to unnecessary diagnostic procedures, treatments, and anxiety due to suspicion of child abuse.
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