TY - JOUR
AU - PhD, Tibor Wittmann, MD,
AB - To the Editor: We read with interest the article by Pazheri et al1 about a pediatric Crohn's disease (CD) case with cutan extraintestinal manifestations and pyostomatitis vegetans (PV). The prevalence rate of oral manifestation in CD varies between 0.5%–20%.2 The majority of children develop more than one type of oral lesion.3 The first cases with PV were observed by Hallopeau in 1898, and the name pyostomatitis vegetans was proposed by McCarthy in 1949 after observing the lesion isolated to the oral cavity. PV is considered the oral equivalent of pyoderma vegetans on the skin.4 PV is relatively frequently associated with inflammatory bowel disease (IBD), mainly with ulcerative colitis (UC), and its prevalence is higher in young adults and in males.5 We report a case of a young man with CD whose combined orofacial symptoms had preceded the severe intestinal penetrating complications by years and, furthermore, the first histological diagnosis was obtained from the oral cavity. A 16-year-old male patient was hospitalized because of diarrhea, anorexia, weight loss, and weakness in 2004. Although he did not agree to endoscopy, on the basis of his clinical symptoms and the laboratory findings of elevated anti-transglutaminase antibody (TTG: 50.3 μ/mL) level and iron deficiency anemia, celiac disease was suspected. On a gluten-free diet the patient had been asymptomatic for 2 years, but then his previous complaints recurred and were accompanied by swelling of the lips and the buccal mucosa, arthralgia, anal skin tags, and an elevated C-reactive protein level of 30.2 mg/L. Small bowel follow-through revealed the thickening of the ileal wall, and leukocyte scintigraphy showed inflammation at the ascending, transverse, and descending colon. In the absence of the patient's consent to the endoscopic examination a buccal biopsy was performed. The histology of the sample showed PV and granuloma, suggesting the presence of CD (Fig. 1). Methylprednisolone treatment was initiated and the patient's oral symptoms resolved. Eight months later an actively draining fistula was observed in the perianal region with symptoms of uvetitis. Rectal ultrasound revealed transsphincteric complex fistula with scrotal abscess. Ileocolonoscopy indicated severely active CD with segmental colonic and anal involvement. The histological examination of the affected part of the bowel confirmed the diagnosis of CD with the presence of epithelioid granulomas. Seton drainage, azathioprine, and antitumor necrosis factor (TNF) therapy (first infliximab, which was switched to adalimumab because of side effects) was initiated. The oral, luminal, and extraintestinal symptoms resolved, the perianal fistula closed, and the discharge ceased completely. Adalimumab was discontinued after 1 year and the patient remained symptom-free over the next 13 months. After this symptom-free period a newly discharging perianal fistula appeared which required repeated adalimumab induction and, subsequently, maintenance therapy which led to prolonged remission again. Figure 1 View largeDownload slide Histology of the buccal biopsy showed pyostomatitis vegetans and granuloma. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.] Figure 1 View largeDownload slide Histology of the buccal biopsy showed pyostomatitis vegetans and granuloma. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.] PV is a rare, chronic mucocutaneous disease of unknown etiology characterized by military pustules that primarily affect the labial ginigival, the buccal, and labial mucosa. These pustules develop across the mucosa and their rupture leads to widespread ulceration.6 Although inflammatory lesions of the oral cavity and ulcerations are more frequently observed in CD than UC, PV seems to be a UC-associated lesion. Theoretically, since granulomas can be found more frequently in the mouth than in any other gastrointestinal tissue in CD, it is possible that finding granulomas hides the concurrence of PV, although, as was seen in our case, PV and orofacial granulomatosis can be present together. The diagnostic value of oral cavity for CD is more important in children, when the disease is less symptomatic, although oral CD is more common. In our case, the abnormality of the buccal mucosa was one of the first signs of the disease and the presence of PV predicted a complicated disease course requiring biological therapy, as in the case of Pazheri et al. References 1 Pazheri F, Alkhouri N, Radhakrishnan K. Pyostomatitis vegetans as an oral manifestation of Crohn's disease in a pediatric patient. Inflamm Bowel Dis . 2010; 16: 2007. CrossRef Search ADS PubMed  2 Rowland M, Fleming P, Bourke B. Looking in the mouth for Crohn's disease. Inflamm Bowel Dis . 2010; 16: 332– 337. CrossRef Search ADS PubMed  3 Pittock S, Drumm B, Fleming P, et al. The oral cavity in Crohn's disease. J Pediatr . 2001; 138: 767– 771. CrossRef Search ADS PubMed  4 Femiano F, Lanza A, Buonaiuto C, et al. Pyostomatitis vegetans: a review of the literature. Med Oral Patol Oral Cir Bucal . 2009; 14: E114– E117. PubMed  5 Hegarty AM, Barrett AW, Scully C. Pyostomatitis vegetans. Clin Exp Dermatol . 2004; 29: 1– 7. CrossRef Search ADS PubMed  6 Markiewicz M, Suresh L, Margarone J, et al. Pyostomatitis vegetans: a clinical marker of silent ulcerative colitis. J Oral Maxillofac Surg . 2007; 65: 346– 348. CrossRef Search ADS PubMed  Copyright © 2011 Crohn's & Colitis Foundation of America, Inc.
TI - Third case: Another pediatric patient with pyostomatitis vegetans and oral granuloma as one of the initial symptoms of Crohn's disease
JO - Inflammatory Bowel Diseases
DO - 10.1002/ibd.21791
DA - 2011-09-01
UR - https://www.deepdyve.com/lp/oxford-university-press/third-case-another-pediatric-patient-with-pyostomatitis-vegetans-and-yED14vo0N6
SP - E122
EP - E123
VL - 17
IS - 9
DP - DeepDyve
ER -