Perianal pseudoverrucous papules and nodules in Hirschsprung's disease: Rapid resolution with oral loperamide

Abstract

We report the case of a 2-year-old boy with Hirschsprung's disease who developed perianal pseudoverrucous papules and nodules subsequent to persistent diarrhea and stool leakage after Giardia infection. Bleeding from the papules resulted in iron deficiency anaemia requiring blood transfusion and iron infusion. Topical therapies used over 6 months were of limited benefit and colostomy was considered, but the condition completely resolved after commencement of oral loperamide. This demonstrates that perianal pseudoverrucous papules and nodules can be severe but are entirely reversible upon removal of the source of skin irritation.

Peristomal umbilicated papules in a child

“…Pseudoverrucous papules and nodules (PPN) is a severe manifestation of chronic skin irritation from urine or feces, usually described in the perianal area as a severe variant of diaper dermatitis. However, cases have been reported in the scrotum, vulva, and around urostomies . It has been described in patients with chronic diarrhea, stool leakage, or continuous dribbling of urine, as seen in Hirschsprung's disease, encopresis, and urinary incontinence due to congenital malformations like spina bifida, hypospadias, and urethral‐vaginal fistulas…”

“…Papules are usually moist and flat topped and can be eroded, friable, and even ulcerated. Bleeding has been reported as a complication, and in severe cases, it can lead to iron deficiency anemia …”

Recurrent Granuloma Gluteale Infantum Secondary to Fecal Overflow Incontinence

“…Treatments that have been used successfully include barrier products, topical and intralesional corticosteroids, and oral loperamide [ 12 - 15 ]. Flurandrenolide-impregnated tape was shown to produce drastic improvements in 2 treated patients [ 13 ].…”

“…However, the use of steroids is controversial as one report found an increase in the size of lesions after steroid use [ 14 ]. Rapid improvement of erosive papulonodular dermatosis can occur if the source of skin irritation can be removed or mitigated [ 15 ]. Our patient experienced rapid resolution and rapid recurrence that corresponded with exposure to liquid stool.…”

Dairy‐free diet improves Jacquet's erosive diaper dermatitis lesions in patients post surgical treatment for Hirschsprung's disease

“…JEDD is a challenging disease, and multiple treatments have been proposed for its cure, however, if incontinence persists, recurrences are frequent. [3][4][5] All our cases were studied to rule out allergy to cow's, sheep's, or goat's milk proteins or lactose intolerance. No proteins neither in blood or feces were found.…”