PERIANAL PAPULO-NODULAR DERMATOSIS IN A H IRSCHSPRUNGS-DISEASE

Introduction. Macerations resulting from anal and/or urinary incontine nce can lead to perineal or peristomal pseudo-verrucosis. The papulo-n odular perianal lesions observed in children after surgery for Hirschs prung syndrome is an example. Case report. A 28-month-old boy with rec tosigmoid Hirschsprung disease developed chronic diarrhea after surger y with cole-anal anastomosis followed rapidly by perineal rash. The ra sh did not respond to local treatments and pseudo-condylomatous papulo -nodules developed. Infection, deficiency and tumoral causes were elim inated. Skin biopsy showed benign epidermal hyperplasia. The lesions r egressed spontaneously within a few weeks after the diarrhea had been controlled and diapers were no longer used, confirming the diagnosis o f perianal papulo-nodular dermatosis following surgical treatment for Hirschprung disease. Discussion. The presentation of the perianal rash was similar to Sevestre and Jacquet dermatitis and infantile gluteal granuloma. The pathogenesis is similar to perianal or peristomal pseud o-verrucosis seen in incontinent or stomy patients. Diagnosis is based on the clinical presentation, the periorificial localization and the pseudo-tumoral aspect of the lesions. Removal of the cause of macerati on, when possible, is the basis of treatment.