[Perianal papulonodular dermatitis in Hirschsprung disease]

Macerations resulting from anal and/or urinary incontinence can lead to perineal or peristomal pseudo-verrucosis. The papulo-nodular perianal lesions observed in children after surgery for Hirschsprung syndrome is an example.

A 28-month-old boy with rectosigmoid Hirschsprung disease developed chronic diarrhea after surgery with colo-anal anastomosis followed rapidly by perineal rash. The rash did not respond to local treatments and pseudo-condylomatous papulonodules developed. Infection, deficiency and tumoral causes were eliminated. Skin biopsy showed benign epidermal hyperplasia. The lesions regressed spontaneously within a few weeks after the diarrhea had been controlled and diapers were no longer used, confirming the diagnosis of perianal papulo-nodular dermatosis following surgical treatment for Hirschprung disease.

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 pseudoverrucosis 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 maceration, when possible, is the basis of treatment.