Although IBD typically presents with gastrointestinal complaints, mucocutaneous lesions have been reported to occur in 15% to 44% of cases and, when present, can precede gastrointestinal symptoms.1,2,3,4 Gregory and Ho5 reviewed the mucocutaneous lesions of IBD and divided them into specific, reactive, and miscellaneous categories (Table 2).
Although uncommon in children, pyoderma gangrenosum is a common mucocutaneous feature of IBD. Typically, the early papulopustules or hemorrhagic bullae rapidly enlarge, become necrotic, and ulcerate. The ulcerations have a characteristic violaceous, undermined border (Fig 4). Lesions can occur on any part of the body but are most frequently located on the lower extremities. Pyoderma gangrenosum is characterized by pathergy (predilection for lesions in areas of trauma); thus, aggressive debridement must be avoided. Twenty percent of adult patients with pyoderma gangrenosum will have associated IBD.6 It is associated more commonly with ulcerative colitis than Crohn's disease, occurring in 5% to 12% and 1% to 2% of patients, respectively.3,5,7 Pyoderma gangrenosum is uncommon in children, with 4% of cases reported in children <15 years old.8 Although IBD is the most common underlying cause of pyoderma gangrenosum in children, pyoderma gangrenosum in children can be associated with several other systemic disorders including immunodeficiencies (primary and HIV related), leukemia, hepatitis, and arthritis.9,10 The occurrence of pyoderma gangrenosum preceding gastrointestinal symptoms in IBD has only been described in a few patients.6,8,11
Perianal lesions including skin tags, fistulas, fissures, and abscesses are characteristic of Crohn's disease and occur during the course of IBD in 60% to 82% of patients; however, 25% to 30% of patients show perianal lesions before gastrointestinal complaints.12,13 The majority of our patients with Crohn's disease (6 of 8), as well as our 1 patient with ulcerative colitis, were noted to have perianal lesions before gastrointestinal symptoms. It is interesting to note that the 2 patients with Crohn's disease who did not have perianal lesions had pyoderma gangrenosum as their initial finding. Of the patients with perianal lesions, in only 2 (patients 8 and 9) was the perianal lesion the presenting complaint. This suggests that perianal lesions may often be present before gastrointestinal complaints but are less often brought to the attention of the physician, especially in adolescent boys.
Although not as well documented, oral lesions associated with IBD are relatively common, occurring in 6% to 20% of patients.14 When present, they are the presenting sign/symptom in approximately one half of cases.1,4,15–1617 In addition, more recent reviews have found an increased prevalence of various oral lesions in IBD, particularly in children. Barnard and Walker-Smith found that 80% of pediatric patients with Crohn's disease and 41% of children with ulcerative colitis had oral lesions in their series of patients.18 In reviews by Pittock et al19 and Plauth et al,4 48% and 66% of patients with Crohn's disease, respectively, were found to have oral manifestations, with an increased prevalence in children.
A variety of specific and nonspecific oral lesions can occur (Table 3). Differences in the percentage of patients with IBD described with oral lesions may relate to specific versus nonspecific oral findings reported. Aphthous ulcers are considered by many to be nonspecific, as they can be seen in up to 20% of the general population; however, aphthae are usually more extensive and persistent when associated with IBD.15 The descriptive term "orofacial granulomatosis" has been used for any granulomatous process of unknown etiology involving the oral cavity,16 which includes disorders previously described as granulomatous cheilitis and partial Melkersson-Rosenthal syndrome. Orofacial granulomatosis is a common manifestation in children with IBD and is typified by recurrent or persistent swelling of the lips, cheeks, gingiva, or oral mucosa with characteristic noncaseating granulomas on histologic examination. Many patients with orofacial granulomatosis do eventually develop gastrointestinal disease consistent with Crohn's disease.20,21 "Cobblestoning" refers to nodular granulomatous swellings that result in a cobblestone appearance of the oral mucosa, particularly on the labial and buccal mucosa. Along with mucosal tags, cobblestoning is highly suggestive of Crohn's disease. Pyostomatitis vegetans, on the other hand, is more characteristic of ulcerative colitis. It is characterized by multiple pustules, erosions, and ulcers on a diffuse erythematous background with vegetations or folding of the gingival and buccal mucosa. Deep, linear ulcers surmounted by hyperplastic folds occur in the gingival sulci and are also specific for IBD.
Genital findings associated with IBD are also more common in Crohn's disease and in children.1 Genital involvement includes vulvar swelling, skin tags, pustules, abscesses, fistulas, fissures, ulcerations, and vaginal discharge.22 Penile and scrotal lesions are less common and include subcoronal ulcers as well as penile and scrotal edema.23 Twenty-five percent of genital Crohn's disease presents before gastrointestinal complaints.1
Although there are few cases reported in the pediatric literature, mucocutaneous lesions presenting as the initial sign of IBD is relatively common and can be an important clue in making the diagnosis of IBD before the development of gastrointestinal symptoms. We have identified 9 pediatric patients with asymptomatic IBD presenting with mucocutaneous lesions. The majority of these patients had oral and/or perianal lesions. Because oral disease, in general, is uncommon in children, we believe that IBD is a common etiology for persistent oral lesions in the pediatric population. Children and adolescents with unexplained oral mucous membrane lesions such as lip/mucosal swelling, gingival hyperplasia, cobblestoning of the oral mucosa, or deep linear ulcerations should have a good history taken regarding gastrointestinal symptoms, fever, and weight loss as well as an examination of the rectal and genital mucosa to seek other mucocutaneous clues of IBD that may not be mentioned to the physician. We would also recommend additional work-up including complete blood count, iron levels, ESR, albumin, occult blood in the stool, and serial endoscopies with biopsies to aid in the diagnosis if suspicions are high.
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К сожалению, для таблиц и фотографии нет места.