There is a very high rate of granuloma detection in oral biopsies, particularly in biopsies of gingival and mucosal tag lesions. This is probably because the oral lesions can be directly visualized and deep biopsies can be undertaken relatively easily. Biopsy of the lower lip is not usually recommended because granulomas are likely to be sparse and scattered in an extensively oedematous lip. There is also the risk of damaging the labial branches of the mental nerve when undertaking a deep biopsy of the lower lip with the consequent risk of long-term paraesthesia or anaesthesia.
Perianal Crohn Disease
As in adults, perianal features can be associated with Crohn disease in children. A recent large study suggests that up to 10% of cases have perianal disease at presentation. The management of perianal Crohn disease is challenging and, as with intestinal disease, needs the involvement of specialized paediatric gastroenterology services [15].
‘Metastatic’ Crohn Disease of the Skin
True metastatic Crohn disease is rare, but is well described in children and can in some cases be the initial presentation in unusual sites such as the vulva (the most common site of metastatic Crohn’s in children) or even the skin of the head and neck. A delay of several years may occur before Crohn disease of the bowel presents in children [16].
Treatment of Systemic Crohn Disease.
The aims of treatment of Crohn disease are to induce and maintain a remission of intestinal inflammation and manage any complications that may develop [17]. Medical therapy is usually the first line of treatment for newly diagnosed disease and for relapses or acute exacerbations. Surgical intervention may be required when there are intractable symptoms despite medical therapy or when there are intestinal complications such as obstruction, perforation, infection, haemorrhage or the development of fistulas. Initial therapy frequently involves the use of the amino salicylic acid (ASA) derivatives sulfasalazine or mesalamine. A high dose of prednisolone followed by a tapering reduction is used to manage acute symptomatic disease. Another anti-inflammatory modality, with an unknown mode of action, is the use of exclusive enteral nutrition. Whole-protein feeds are as effective as elemental feeds. This form of treatment is almost as effective as prednisolone for inducing remission and is particularly attractive for use in children because of the lack of toxicity and potential to enhance nutrition and growth.
The steroid-sparing immunosuppressant agent 6-mercaptopurine (a metabolite of azathioprine) may be used to control the inflammatory component of the disease and the antibacterials metronidazole or ciprofloxacillin may be prescribed to alter the intestinal flora to reduce inflammation. In more recalcitrant cases, biological agents such as infliximab or adalimumab may be administered.
References
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13 Neville BW, Smith SE, Maize JC et al. Pyostomatitis vegetans. Am J Dermatopathol 1985;7:69–77.
14 Ficarra G, Cicchi P, Amorosi A et al. Oral Crohn’s disease and pyostomatitis vegetans. An unusual association. Oral Surg Oral Med Oral Pathol 1993;75:220–4.
15 Keljo DJ, Markowitz J, Langton C et al. Course and treatment of perianal disease in children newly diagnosed with Crohn’s disease. Inflamm Bowel Dis 2009;15(3):383–7.
16 Palamaras I, El-Jabbour J, Pietropaolo N et al. Metastatic Crohn’s disease: a review. Eur Acad Dermatol Venereol 2008;22(9):1033–43.
17 Egan LJ, Sandborn WJ. Advances in the treatment of Crohn’s disease. Gastroenterology 2004;126:1574–81.
Orofacial granulomatosis
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