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Extraintestinal Crohn's disease: Case report and review of the literature.


Crohn 's disease is a granulomatous inflanmatory bowel disease. Its pathologic findings include noncontiguous chronic inflammation and noncaseating granulomas. Any segment of the gastrointestinal tract can be involved, but it is uncommon to find that Crohn's disease has spread beyond the intestine. We describe the case of a man with extra intestinal Crohn's disease that was marked by quiescent involvement of the lower gastrointestinal tract and florid involvement of the nasal cavity, supraglottic structures, glottis, and skin.


Crohn's disease is classically described as a chronic granulomatous inflammatory bowel disease. Its pathologic findings include noncontiguous chronic inflammation and noncaseating granulomas. Any segment of the gastrointestinal tract can be involved in the disease process, but it is unusual for the disease to extend into the upper aerodigestive system. In this article, we describe a case of extraintestinal Crohn's disease that featured quiescent involvement of the lower gastrointestinal tract and florid involvement of the nasal cavity, supraglottic structures, glottis, and skin.

Case report

A 45-year-old black man with known Crohn's disease came to the office for an evaluation of nasal obstruction. He had originally been diagnosed with Crohn's disease in 1982; he had been treated with steroids at the time, which had adequately controlled his symptoms. During his visit to our office, the patient reported that his symptoms of nasal obstruction had initially manifested in 1985. He was found then to have a bilateral nasal stenosis; he also had pyoderma of the scalp. Pathologic tissue evaluation revealed an inflammatory process. He underwent mucosal debridement and nasal stenting.

The patient was asymptomatic for 4 years. But in 1989, he again began to experience nasal obstruction along with intermittent epistaxis. At that time he underwent laser therapy to clear his nasal passages. At no time had the patient experienced any exacerbation of his intestinal Crohn's disease. In 1991, the patient was found to have a restenosis of his nasal passages along with laryngeal edema and hoarseness. An attempt to resurface the nasal mucosa with a buccal graft failed. He was started on 6mercaptopurine.

In January 1997, we used a CO [2] laser to open his nasal passages, and we performed direct laryngoscopy and tracheoscopy to assess his upper airway. Our examination of his supraglottic structures revealed the presence of edematous tissue with areas of granulation that involved his epiglottis and false vocal folds. Biopsies taken of the posterior cricoid region and epiglottis were consistent with Crohn's disease. Postoperatively, we doubled the dosage of his 6-mercaptopurine, which stabilized his nasal and laryngeal disease. However, repeat laryngoscopy revealed a persistent involvement of his laryngeal structures.


Our patient had experienced both gastrointestinal and extraintestinal manifestations of Crohn's disease. His disease involved the small and large intestines, scalp, anus, and upper respiratory tract (nasal cavity, nasal pharynx, hypopharynx, and larynx).

As noted by Greenstein et al, 36% of patients with Crohn's disease have extraintestinal involvement.[1] More than 40% of these patients with extraintestinal involvement experience cutaneous manifestations.[1]

Involvement of the aerodigestive tract has been documented in the literature, and oral manifestations of Crohn's disease have been noted to precede its gastrointestinal appearance in 26 to 60% of cases.[2-6] Involvement of the esophagus has been reported to occur in fewer than 2% of cases,[7] and isolated cases of laryngeal4-6 and nasal8-0 involvement have been documented (table).

Oral involvment. Oral involvement has been reported to manifest as a diffuse swelling of the lips that is associated with pain, cobblestoning of the buccal mucosa, and painful ulcerations with hyperplastic margins. [2] The differential diagnosis includes orofacial granulomatosis, sarcoidosis, tuberculosis, Wegener's syndrome, and sinonasal lymphoma. On pathologic analysis, the diagnosis can be ascertained by observing the presence of chronic inflammatory changes with noncaseating granulomas and Langhans'-type giant cells.

Nasal involvement. There have been three cases of nasal Crohn's disease documented in the literature. [8-10] Kinnear described the case of a 36-year-old woman with chronic nasal congestion, crusting, and inflamed nasopharyngeal tissue in the setting of known Crohn's disease. [8] Ernst et al described the case of a 17-year-old girl who had pansinusitis with polypoidal changes. [9] Biopsies of these lesions revealed tissue changes that were consistent with Crohn's disease. The patient was treated with oral steroids in a tapered manner, and her symptoms resolved. Finally, Pochon et al described a case in which a 38-year-old man with known Crohn's disease had a bilateral nasal obstruction and watery discharge. [10] Physical examination revealed that tissue changes involved the septal mucosa on the left and that the nasal mucosa was swollen to the point that it had completely obstructed the nasal passages. The patient underwent an evacuation of the septal edema, which released approximately 1.5 ml of clear fluid. He was started on intravenous steroids and later switched to oral steroids on a tapered schedule. This resulted in a complete resolution of the clinical findings.

Laryngeal involvement. Croft and Wilkinson reported a case of laryngeal involvement of Crohn's disease that resolved with the use of oral steroids. [4] Kelly et al reported two cases of Crohn's involvement of the larynx, both of which improved with oral steroids. [5]

The case of our patient is unusual because the clinical manifestations of his Crohn's disease have not been previously reported. [1-11] Thus far, the aggressive form of this patient's disease has failed to respond to standard medical management with systemic and topical steroids and 6-mercaptopurine. Even with surgical intervention, we have only been able to slow the process. Since performing surgery and increasing the 6-mercaptopurine dosage, we have been able to lengthen the interval between the patient's laser treatments to every 9 to 12 months. Even so, we are significantly concerned about the progressive laryngeal involvement, which remains a difficult management problem. This very aggressive manifestation of Crohn's disease can be classified technically as metastatic Crohn's disease because of its intestinal and extraintestinal involvement. [11] This case demonstrates the need to be aware of extraintestinal Crohn's disease and to be proactive in the management of these patients.

From the Great Lakes Naval Health Facility, Chicago (Dr. Ulnick), and the Division of Otolaryngology, Children's Hospital and Regional Medical Center, Seattle (Dr. Perkins).

Reprint requests: Jonathan Perkins, DO, Division of Otolaryngology, Children's Hospital, 4800 Sandpoint Way N.E., CH-62, Seattle, WA 98105. Phone: (206) 526-2100, ext. 3864; fax: (206) 5273878.


(1.) Greenstein AJ, Janowitz HD, Sachar DB. The extra-intestinal complications of Crohn's disease and ulcerative colitis: A study of 700 patients. Medicine (Baltimore) 1976;55:401-12.

(2.) Cleary KR, Batsakis JG. Orofacial granulomatosis and Crohn's disease. Ann Otol Rhinol Laryngol 1996;105:166-7.

(3.) Misra S. Ament ME. Orofacial lesions in Crohn's disease. Am J Gastroenterol 1996;91:1651-3.

(4.) Croft CB, Wilkinson AR. Ulceration of the mouth, pharynx, and larynx in Crohn's disease of the intestine. Br J Surg 1972;59:249-52.

(5.) Kelly JH, Montgomery WW, Goodman ML, Mulvaney TJ. Upper airway obstruction associated with regional enteritis. Ann Otol Rhinol Laryngol 1979;88:95-9.

(6.) Wilder WM, Slagle GW, Hand AM, Watkins WJ. Crohn's disease of the epiglottis, aryepiglottic folds, anus, and rectum. J Clin Gastroenterol 1980;2:87-91.

(7.) Walker RS, Breuer RI. Victor T, Gore RM. Crohn's esophagitis: A unique cause of esophageal polyposis. Gastrointest Endose 1996;43:511-5.

(8.) Kinnear WJ. Crohn's disease affecting the nasal mucosa. J Otolaryngol 1985;14:399-400.

(9.) Ernst A, Preyer S, Plauth M, Jenss H. [Polypoid pansinusitis in an unusual, extra-intestinal manifestation of Crohn disease]. HNO 1993;41:33-6.

(10.) Pochon N, Dulguerov P. Widgren S. Nasal manifestations of Crohn's disease. Otolaryngol Head Neck Surg 1995;113:813-5.

(11.) Chen W, Blume-Peytavi U, Goerdt S, Orfanos CE. Metastatic Crohn's disease of the face. J Am Acad Dermatol 1996;35:986-8.
Table. Characteristics of reported cases of nasal or laryngeal
manifestations of Crohn's disease
Author Age * Sex Symptoms Gl findings
Croft and 27 M Dysphagia Positive Hx+
1972 4
Kelly et al, 25 M Odynophagia, Positive Hx
1979 5 dyspnea,
 dysphagia, and
 22 F Dysphagia, Positive Hx
 voice changes,
 nocturnal dyspnea,
 and stridor
Wilder et al, 23 F Sore throat and Negative Hx
1980 6 breathing difficulty
Kinnear, 36 F Chronic nasal Positive Hx
1985 8 congestion
Ernst et al, 17 F Nasal congestion, Positive Hx
1993 9 drainage, and
 sore throat
Pochon et al, 38 M Nasal congestion Positive Hx
1995 10
Ulnick and 45 M Nasal obstruction Positive Hx
Author Physical examination Treatment
Croft and Ulcerations of the Steroids
Wilkinson, buccal mucosa and
1972 4 palate,edema,
 erythema, and
 thickened epiglottis,
 aryepiglottic folds,
 arytenoids, and false
 vocal folds
Kelly et al, Edema, erythema, and Prednisone
1979 5 thickened epiglottis,
 aryepiglottic folds,
 arytenoids, and false
 vocal folds
 Edema, erythema, and Tracheoscopy (failed),
 thickened epiglottis, followed by steroids
 aryepiglottic folds,
 arytenoids, and false
 vocal folds
Wilder et al, Edema, erythema, and Metronidazole,
1980 6 thickened epiglottis hydrocortisone,
 and aryepiglottic folds and sulfasalazine
Kinnear, Atrophic rhinitis and Topical steroids
1985 8 hypertrophic
 nasopharyngeal tissue
Ernst et al, Polypoid pansinusitis Steroids
1993 9 and peritonsillitis
Pochon et al, Edematous nasal Steroids
1995 10 mucosa obstucting
 the nasal cavity
Ulnick and Edematous epiglottis Laser surgery,
Perkins, and false vocal folds, systemic and
2000ss with areas of topical steroids,
 granulation 6-mercaptopurine
(*)Age in years.
(+)History of Crohn's disease.
(ss)Present study.
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Comment:Extraintestinal Crohn's disease: Case report and review of the literature.
Author:Perkins, Jonathan
Publication:Ear, Nose and Throat Journal
Geographic Code:1USA
Date:Feb 1, 2001
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