Infantile Crohn Disease Presenting with Diarrhea and Pyoderma Gangrenosum

Abstract

Cutaneous erosions and ulcerations in the diaper area are common in infancy and usually result from local irritation. We describe an infant with chronic diarrhea and failure to thrive who developed extensive ulcerations in the inguinal folds and perineum that were initially thought to be exclusively caused by local irritation. A cutaneous examination found signs consistent with those of pyoderma gangrenosum, leading to a diagnosis of infantile Crohn disease. Cutaneous signs can lead to the diagnosis of an underlying systemic disease in infants with chronic diarrhea and rash. Prompt diagnosis is especially important in infantile Crohn disease, since many infants require surgical resection of affected bowel, and 60% die from disease complications. This article reports a rare instance of an infant who developed pyoderma gangrenosum due to Crohn disease and reviews cutaneous signs of systemic disease in infants presenting with chronic diarrhea and rash.

Infantile pyoderma gangrenosum

“…6,9 Other systemic treatments included dapsone, 2,3,6 colchicine, 3 azathioprine, 3 sulfasalazine, and 6-mercaptopurine. 9 Topical treatments included steroid (3 patients) and tacrolimus (one patient). Intralesional steroids were used in 3 patients (Table I).…”

“…Systemic therapy is usually required to control the disease in this age group, evidenced by the fact that 11 of the 12 reported infants with PG had treatment with systemic steroids. [2][3][4][5][6][7][9][10][11][12][13] Eight were treated with oral steroid, 2-5,10-13 one with intravenous steroid, 7 and two with both intravenous and oral steroids. 6,9 Other systemic treatments included dapsone, 2,3,6 colchicine, 3 azathioprine, 3 sulfasalazine, and 6-mercaptopurine.…”

“…8 A further infant had Crohn's disease. 9 The diagnosis of PG in infants is established (as in all age groups) by the clinical presentation, exclusion of alternative diagnoses (especially infectious or malignant processes), and skin biopsy specimens showing the histopathologic changes consistent with PG. Most of the reported cases of infantile PG have had their disease successfully controlled with systemic steroids.…”

“…It has been estimated that 4% of PG cases are in infants and children 1 and, to our knowledge, there are only 12 cases reported in the literature of PG in infants (\1 year of age). [2][3][4][5][6][7][8][9][10][11][12][13] Eight of the reported patients with infantile PG had an underlying medical condition (Table I). Two infants had Takayasu's arteritis, 2,3 one of whom also had sterile osteomyelitis 3 and one further patient had chronic recurrent multifocal osteomyelitis.…”

Pyoderma gangrenosum in an infant: A case report and review of the literature

“…It is often associated with a concomitant disease, although up to half of cases are considered idiopathic . Infantile PG is even less common, with only 20 cases reported, 11 of which are described as idiopathic, whereas the others are associated with a spectrum of inflammatory, hematologic, and infectious disorders (Table ) . With this report, we add another patient with infantile PG and review the literature for potential treatments.…”