Crohn's disease presenting as palatal ulcer
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《美国医学杂志》
Kanchi Kamakoti CHILDS Trust Hospital, Chennai, India
Abstract
Crohn's disease (CD) in children younger than 5 years of age is termed as early onset inflammatory bowel disease (EO-IBD). We report a 4 yr 6 mo-old child with EO-IBD, who presented with palatal ulcer, an extra intestinal manifestation of Crohn's disease as the dominant feature.
Keywords: Crohn′s disease; EO-IBD; Children; Steroids
Crohn's disease (CD) is a chronic inflammatory bowel disease characterized by transmural granulomatous inflammation involving any part of the gastrointestinal tract in a discontinuous manner. The incidence of pediatric CD in US varies from 0.2-8.5 per 1,00,000, and children less than 5 years of age constitute 4% of the pediatric CD.[1] This "early onset IBD (EO-IBD)" has been recognized as a special subset of IBD[2] because of its great potential to affect the growth and development. A recent study from Chennai had reported the clinical manifestations of Crohn's disease in children and adolescents.[3] This case report is to highlight the unusual age and presentation of Crohn's disease.
Case report
A 4-year-6 month-old-boy presented with fever, palatal ulcer and odynophagia of one-month duration. He also had crampy abdominal pain associated with passage of loose stools, occasionally tinged with blood for 2 weeks with loss of weight. There was no history of arthralgia, rash, pedal edema, jaundice or contact with tuberculosis.
On examination, he was febrile, weighed 17 kg and his height was 102 cms. An irregular ulcer 1x2 cm with raised margins was seen on the soft palate. There was no significant lymphadenopathy. General and systems examination were normal.
Investigations showed a Hb of 11.9 gm/dl; leukocyte count was 10,900 cells/cu. mm with neutrophilic predominance. Reactive protein was positive 96 mg/dl and erythrocyte sedimentation rate (ESR) was 40 mm at 1 hour. Chest skiagram and ultrasound abdomen were normal. Antinuclear antibodies, antibodies to HIV and herpes simplex viral antigens were negative. Liver function tests (LFT) and renal function tests (RFT) were normal. Cultures of blood, urine and bone marrow showed no growth. Upper GI endoscopy showed erythematous antral gastritis, and biopsy revealed focal active granulomatous gastritis Figure1. Ileocolonoscopy showed multiple aphthous ulcers in sigmoid, descending and transverse colon. Rectum and terminal ileum were normal. Biopsy from these lesions revealed non-caseating granulomas, consistent with Crohn's disease Figure2. Biopsy from oral ulcer was not taken; however, smear for AFB and fungal elements were negative. A diagnosis of early onset Crohn's disease with moderate to severe activity was made. The child was treated with IV methyl prednisolone, followed by oral prednisolone 2 mg/kg/day, tapered over 6 weeks and stopped. Mesalamine 50 mg/kg/day was also started. Supplemental enteral nutrition was advised for one week, followed by an adequate calorie home-made diet. On initiating therapy, his fever settled within 48 hours, appetite improved, stools became normal; the ulcer healed in 4 days and he gained 2 kg weight in one month. This child is being presented for the uncommon age and unusual mode of presentation of Crohn's disease.
Discussion
Inflammatory Bowel Disease (IBD) is an important cause of chronic morbidity in childhood and adolescence[4]. Crohn's disease (CD) and ulcerative colitis (UC) are the two important entities of IBD. The median age of presentation of CD is 7.9 years, and 4% of pediatric CD occurs below the age of 5.[1] This is probably the youngest case of CD reported from India. The pathogenesis of CD is still unclear and most likely due to interplay of genetic, environmental and immune factors. The CD susceptible gene NOD2/CARD 15 (Caspase Activation Recruitment Domain) has been identified on chromosome 16 and mutations in this region is associated more with EO-IBD.[5] The common gastrointestinal manifestations are abdominal pain, diarrhea, fever and weight loss.[6] The location and extent of disease involvement of the gastrointestinal tract determine the clinical presentation of CD. Extra-intestinal manifestations are seen in 25-35% of children with IBD and may outweigh those related to the GIT by several years. Recurrent aphthous ulcer is the most common oral manifestation of IBD and is a frequent finding in CD. The other disease-specific oral manifestations include mucosal tags, lip swelling, fissures, buccal mucosal swelling or cobble stoning, deep linear ulcerations and localised mucogingivitis.[7] This child had only a palatal ulcer and none of the reported lesions. The oral lesions being directly visible is more accessible for tissue diagnosis. However, if other sites show a characteristic granuloma, then oral biopsy is not clinically justified.[7] The diagnosis of CD is by a combination of clinical, laboratory, endoscopic and histological findings. The rectum is usually spared in CD and the inflammation is asymmetric with deep, irregular ulcers surrounded by normal looking mucosa. Focal enhanced gastritis and identification of microgranuloma on antral biopsy has also helped in increasing the diagnostic yield.[8] The role of serological assay in the diagnostic armamentarium of IBD is not very significant. The goals of treatment for children with CD are to achieve the best clinical and laboratory control with least possible side effects from medications, to promote growth through adequate nutrition, and to permit the child to function as normal as possible. The medical management of CD consists of 5-aminosalicylic acid (5ASA) with or without steroids depending on the activity of the disease. Immunosuppressive drugs like azathioprine, 6 mercaptopurine, cyclosporin A and methotrexate are used when there is failure to respond to corticosteroid or if the child is steroid-dependent.[9] Infliximab has proved effective in short-term treatment of moderate to severe CD in children. Nutritional therapy is an important modality for treatment during disease activity and growth failure. Surgery is indicated in the presence of obstruction, hemorrhage or perforation. CD in children unlike in adults has a significant impact on its nutrition, growth and development in addition to the psychological stress.[10] It is therefore essential that an early diagnosis is made and appropriate treatment is initiated. The total management of a child with CD is a multidisciplinary approach involving the pediatrician, pediatric gastroenterologist, nutritionist, psychologist and surgeon.
References
1. Baldassano RN, Piccoli DA. Inflammatory bowel disease in pediatric and adolescent patients. Gastroenterol Clin North Am 1999; 28: 445-455.
2. Mamula P, Telega GW, Markowitz JE, Brown KA, Russo PA, Piccoli DA et al. Inflammatory bowel disease in children 5 years of age and younger. Am J Gastroenterol 2002; 97 : 2005-2010.
3. Malathi S, BhaskarRaju B, Shivbalan So, Rajarajan K. Pediatic Crohn's Disease in South India. Indian Pediatr 2005; 42 : 459-463.
4. Cuffari C, Darbari A. Inflammatory bowel disease in the pediatric and adoloscent patient. Gastroenterol Clin N Am 2002; 31 : 275-291.
5. Bonen DK, Cho JK. The genetics of inflammatory bowel disease. Gastroenterol 2003; 124 : 521-536.
6. Hyams JS. Crohn's disease. In Wyllie R, Hyams JS, eds. Pediatric Gastrointestinal Disease: Pathophysiology, Diagnosis, Management. Philadelphia; WB Saunders 1999:401-18.
7. Pittock S, Drumm B, Fleming P, Mc Dermott M, Imrie C, Flint S et al. The oral cavity in Crohn's disease. J Pediatr 2002;138(5) : 767-771.
8. Abdullah BA, Gupta SK, Croffie JM, Pfefferkorn MD, Molleston JP, Corkins MR et al. The role of oesophagoduodenoscopy in the initial evaluation of childhood inflammatory bowel disease: A 7 year study. J Pediatr Gastroenterol 2002; 35; 636-640.
9. Markowitz J, Grancher K, Kohn N, Daum F. Immunomodulatory therapy for pediatric inflammatory bowel disease : changing patterns of use, 1999-2000. Am J Gastroenterol 2002; 97(4): 928-932.
10. Mamula P, Markowitz JE, Baldassano RN. Inflammatory bowel disease in early childhood and adoloscence:special considerations. Gastroenterol clin North Am 2003; 32 : 967-995.(Ganesh R, Suresh N, Ezhil)
Abstract
Crohn's disease (CD) in children younger than 5 years of age is termed as early onset inflammatory bowel disease (EO-IBD). We report a 4 yr 6 mo-old child with EO-IBD, who presented with palatal ulcer, an extra intestinal manifestation of Crohn's disease as the dominant feature.
Keywords: Crohn′s disease; EO-IBD; Children; Steroids
Crohn's disease (CD) is a chronic inflammatory bowel disease characterized by transmural granulomatous inflammation involving any part of the gastrointestinal tract in a discontinuous manner. The incidence of pediatric CD in US varies from 0.2-8.5 per 1,00,000, and children less than 5 years of age constitute 4% of the pediatric CD.[1] This "early onset IBD (EO-IBD)" has been recognized as a special subset of IBD[2] because of its great potential to affect the growth and development. A recent study from Chennai had reported the clinical manifestations of Crohn's disease in children and adolescents.[3] This case report is to highlight the unusual age and presentation of Crohn's disease.
Case report
A 4-year-6 month-old-boy presented with fever, palatal ulcer and odynophagia of one-month duration. He also had crampy abdominal pain associated with passage of loose stools, occasionally tinged with blood for 2 weeks with loss of weight. There was no history of arthralgia, rash, pedal edema, jaundice or contact with tuberculosis.
On examination, he was febrile, weighed 17 kg and his height was 102 cms. An irregular ulcer 1x2 cm with raised margins was seen on the soft palate. There was no significant lymphadenopathy. General and systems examination were normal.
Investigations showed a Hb of 11.9 gm/dl; leukocyte count was 10,900 cells/cu. mm with neutrophilic predominance. Reactive protein was positive 96 mg/dl and erythrocyte sedimentation rate (ESR) was 40 mm at 1 hour. Chest skiagram and ultrasound abdomen were normal. Antinuclear antibodies, antibodies to HIV and herpes simplex viral antigens were negative. Liver function tests (LFT) and renal function tests (RFT) were normal. Cultures of blood, urine and bone marrow showed no growth. Upper GI endoscopy showed erythematous antral gastritis, and biopsy revealed focal active granulomatous gastritis Figure1. Ileocolonoscopy showed multiple aphthous ulcers in sigmoid, descending and transverse colon. Rectum and terminal ileum were normal. Biopsy from these lesions revealed non-caseating granulomas, consistent with Crohn's disease Figure2. Biopsy from oral ulcer was not taken; however, smear for AFB and fungal elements were negative. A diagnosis of early onset Crohn's disease with moderate to severe activity was made. The child was treated with IV methyl prednisolone, followed by oral prednisolone 2 mg/kg/day, tapered over 6 weeks and stopped. Mesalamine 50 mg/kg/day was also started. Supplemental enteral nutrition was advised for one week, followed by an adequate calorie home-made diet. On initiating therapy, his fever settled within 48 hours, appetite improved, stools became normal; the ulcer healed in 4 days and he gained 2 kg weight in one month. This child is being presented for the uncommon age and unusual mode of presentation of Crohn's disease.
Discussion
Inflammatory Bowel Disease (IBD) is an important cause of chronic morbidity in childhood and adolescence[4]. Crohn's disease (CD) and ulcerative colitis (UC) are the two important entities of IBD. The median age of presentation of CD is 7.9 years, and 4% of pediatric CD occurs below the age of 5.[1] This is probably the youngest case of CD reported from India. The pathogenesis of CD is still unclear and most likely due to interplay of genetic, environmental and immune factors. The CD susceptible gene NOD2/CARD 15 (Caspase Activation Recruitment Domain) has been identified on chromosome 16 and mutations in this region is associated more with EO-IBD.[5] The common gastrointestinal manifestations are abdominal pain, diarrhea, fever and weight loss.[6] The location and extent of disease involvement of the gastrointestinal tract determine the clinical presentation of CD. Extra-intestinal manifestations are seen in 25-35% of children with IBD and may outweigh those related to the GIT by several years. Recurrent aphthous ulcer is the most common oral manifestation of IBD and is a frequent finding in CD. The other disease-specific oral manifestations include mucosal tags, lip swelling, fissures, buccal mucosal swelling or cobble stoning, deep linear ulcerations and localised mucogingivitis.[7] This child had only a palatal ulcer and none of the reported lesions. The oral lesions being directly visible is more accessible for tissue diagnosis. However, if other sites show a characteristic granuloma, then oral biopsy is not clinically justified.[7] The diagnosis of CD is by a combination of clinical, laboratory, endoscopic and histological findings. The rectum is usually spared in CD and the inflammation is asymmetric with deep, irregular ulcers surrounded by normal looking mucosa. Focal enhanced gastritis and identification of microgranuloma on antral biopsy has also helped in increasing the diagnostic yield.[8] The role of serological assay in the diagnostic armamentarium of IBD is not very significant. The goals of treatment for children with CD are to achieve the best clinical and laboratory control with least possible side effects from medications, to promote growth through adequate nutrition, and to permit the child to function as normal as possible. The medical management of CD consists of 5-aminosalicylic acid (5ASA) with or without steroids depending on the activity of the disease. Immunosuppressive drugs like azathioprine, 6 mercaptopurine, cyclosporin A and methotrexate are used when there is failure to respond to corticosteroid or if the child is steroid-dependent.[9] Infliximab has proved effective in short-term treatment of moderate to severe CD in children. Nutritional therapy is an important modality for treatment during disease activity and growth failure. Surgery is indicated in the presence of obstruction, hemorrhage or perforation. CD in children unlike in adults has a significant impact on its nutrition, growth and development in addition to the psychological stress.[10] It is therefore essential that an early diagnosis is made and appropriate treatment is initiated. The total management of a child with CD is a multidisciplinary approach involving the pediatrician, pediatric gastroenterologist, nutritionist, psychologist and surgeon.
References
1. Baldassano RN, Piccoli DA. Inflammatory bowel disease in pediatric and adolescent patients. Gastroenterol Clin North Am 1999; 28: 445-455.
2. Mamula P, Telega GW, Markowitz JE, Brown KA, Russo PA, Piccoli DA et al. Inflammatory bowel disease in children 5 years of age and younger. Am J Gastroenterol 2002; 97 : 2005-2010.
3. Malathi S, BhaskarRaju B, Shivbalan So, Rajarajan K. Pediatic Crohn's Disease in South India. Indian Pediatr 2005; 42 : 459-463.
4. Cuffari C, Darbari A. Inflammatory bowel disease in the pediatric and adoloscent patient. Gastroenterol Clin N Am 2002; 31 : 275-291.
5. Bonen DK, Cho JK. The genetics of inflammatory bowel disease. Gastroenterol 2003; 124 : 521-536.
6. Hyams JS. Crohn's disease. In Wyllie R, Hyams JS, eds. Pediatric Gastrointestinal Disease: Pathophysiology, Diagnosis, Management. Philadelphia; WB Saunders 1999:401-18.
7. Pittock S, Drumm B, Fleming P, Mc Dermott M, Imrie C, Flint S et al. The oral cavity in Crohn's disease. J Pediatr 2002;138(5) : 767-771.
8. Abdullah BA, Gupta SK, Croffie JM, Pfefferkorn MD, Molleston JP, Corkins MR et al. The role of oesophagoduodenoscopy in the initial evaluation of childhood inflammatory bowel disease: A 7 year study. J Pediatr Gastroenterol 2002; 35; 636-640.
9. Markowitz J, Grancher K, Kohn N, Daum F. Immunomodulatory therapy for pediatric inflammatory bowel disease : changing patterns of use, 1999-2000. Am J Gastroenterol 2002; 97(4): 928-932.
10. Mamula P, Markowitz JE, Baldassano RN. Inflammatory bowel disease in early childhood and adoloscence:special considerations. Gastroenterol clin North Am 2003; 32 : 967-995.(Ganesh R, Suresh N, Ezhil)