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Read My Lips: Oral Manifestations of Systemic Diseases
Read My Lips: Oral Manifestations of Systemic Diseases
A
14-year-old boy presented with a 6-month history of painful swelling of his lips (Figures 1 and 2). The swelling was initially intermittent, but became progressive and persistent. It began on his upper lip and gradually extended to the lower lip. A complete blood count showed anemia (hemoglobin 9 g/dL), and iron supplementation was started. The patient developed abdominal pain, and he lost 7 kg within a month. On presentation to a dermatologist, prick tests were performed, which were negative. Serum C1 esterase inhibitor and angiotensin-converting enzyme levels were normal. A Mantoux test was negative. Gastroscopy and colonoscopy performed to rule out gastrointestinal bleeding revealed patchy erythema, mucosal friability, thickened folds, and ulcerations in the esophagus, jejunum, and colon (Figure 3). Analysis of multiple biopsy specimens obtained from the affected areas demonstrated periodic acid-Schiff-negative noncaseating granulomas, confirming the diagnosis of Crohn’s disease. A punch biopsy specimen of the buccal mucosa showed noncaseating granulomatous inflammation, confirming the clinical impression of granulomatous cheilitis secondary to inflammatory bowel disease. Granulomatous cheilitis is a chronic swelling of the lip due to granulomatous inflammation. It is a rare condition, although its actual frequency is unknown. Onset is most common in young adulthood. Morbidity related to granulomatous cheilitis
Figure 2. Fissuring in the midline of the lips due to enlargement of the lips.
Figure 1. Erythematous swelling involving the upper lip and the lower lip. J Pediatr 2013;163:1784-5. 0022-3476/$ - see front matter. Copyright ª 2013 Mosby Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2013.06.080
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Figure 3. Patchy erythema, mucosal friability, thickened folds, and ulcerations in the esophagus, jejunum, and colon.
Vol. 163, No. 6 December 2013 depends on whether an underlying organic disease, such as inflammatory bowel disease1 or sarcoidosis, is present. Granulomatous cheilitis is a recognized early manifestation of Crohn’s disease.2 The patient was started on oral corticosteroids and mesalazine. This regimen produced prompt remission of the Crohn’s disease exacerbation, but slow improvement of the lip swelling. n Marıa Jose de Castro L opez, MD Laura Illade Quinteiro, MD Federico Martin on Torres, PhD
Juan Manuel Cutrın Prieto, MD Department of Pediatrics Hospital of the University of Santiago of Compostela Galicia, Spain
References 1. Sanderson J, Nunes C, Escudier M, Barnard K, Shirlaw P, Odell E, et al. Oro-facial granulomatosis: Crohn’s disease or a new inflammatory bowel disease? Inflamm Bowel Dis 2005;11:840-6. 2. Rowland M, Fleming P, Bourke B. Looking in the mouth for Crohn’s disease. Inflamm Bowel Dis 2010;16:332-7.
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A
14-year-old boy presented with a 6-month history of painful swelling of his lips (Figures 1 and 2). The swelling was initially intermittent, but became progressive and persistent. It began on his upper lip and gradually extended to the lower lip. A complete blood count showed anemia (hemoglobin 9 g/dL), and iron supplementation was started. The patient developed abdominal pain, and he lost 7 kg within a month. On presentation to a dermatologist, prick tests were performed, which were negative. Serum C1 esterase inhibitor and angiotensin-converting enzyme levels were normal. A Mantoux test was negative. Gastroscopy and colonoscopy performed to rule out gastrointestinal bleeding revealed patchy erythema, mucosal friability, thickened folds, and ulcerations in the esophagus, jejunum, and colon (Figure 3). Analysis of multiple biopsy specimens obtained from the affected areas demonstrated periodic acid-Schiff-negative noncaseating granulomas, confirming the diagnosis of Crohn’s disease. A punch biopsy specimen of the buccal mucosa showed noncaseating granulomatous inflammation, confirming the clinical impression of granulomatous cheilitis secondary to inflammatory bowel disease. Granulomatous cheilitis is a chronic swelling of the lip due to granulomatous inflammation. It is a rare condition, although its actual frequency is unknown. Onset is most common in young adulthood. Morbidity related to granulomatous cheilitis
Figure 2. Fissuring in the midline of the lips due to enlargement of the lips.
Figure 1. Erythematous swelling involving the upper lip and the lower lip. J Pediatr 2013;163:1784-5. 0022-3476/$ - see front matter. Copyright ª 2013 Mosby Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2013.06.080
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Figure 3. Patchy erythema, mucosal friability, thickened folds, and ulcerations in the esophagus, jejunum, and colon.
Vol. 163, No. 6 December 2013 depends on whether an underlying organic disease, such as inflammatory bowel disease1 or sarcoidosis, is present. Granulomatous cheilitis is a recognized early manifestation of Crohn’s disease.2 The patient was started on oral corticosteroids and mesalazine. This regimen produced prompt remission of the Crohn’s disease exacerbation, but slow improvement of the lip swelling. n Marıa Jose de Castro L opez, MD Laura Illade Quinteiro, MD Federico Martin on Torres, PhD
Juan Manuel Cutrın Prieto, MD Department of Pediatrics Hospital of the University of Santiago of Compostela Galicia, Spain
References 1. Sanderson J, Nunes C, Escudier M, Barnard K, Shirlaw P, Odell E, et al. Oro-facial granulomatosis: Crohn’s disease or a new inflammatory bowel disease? Inflamm Bowel Dis 2005;11:840-6. 2. Rowland M, Fleming P, Bourke B. Looking in the mouth for Crohn’s disease. Inflamm Bowel Dis 2010;16:332-7.
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