Etiology • A granulomatous disease of unknown etiology • Genetic factors coupled with environmental influences appear
of greatest importance
Clinical Presentation • Extraintestinal/oral findings may include the following:
• Nodular submucosal nodules of lips (granulomas) • Polypoid masses with fissures and ulceration along the
buccal/labial sulcus • Oral ulcers of nonspecific/aphthous type may develop. • May present as orofacial granulomatosis such as
granulomatous cheilitis • Lesions of pyostomatitis vegetans may be associated.
of greatest importance
Clinical Presentation • Extraintestinal/oral findings may include the following:
• Nodular submucosal nodules of lips (granulomas) • Polypoid masses with fissures and ulceration along the
buccal/labial sulcus • Oral ulcers of nonspecific/aphthous type may develop. • May present as orofacial granulomatosis such as
granulomatous cheilitis • Lesions of pyostomatitis vegetans may be associated.
Diagnosis • Correlation of mucosal lesions with intestinal symptoms of
cramping, diarrhea, and associated weight loss • Oral mucosal biopsy results demonstrate noncaseating, epi-
theloid granulomas within submucosa
Differential Diagnosis • Deep fungal diseases including blastomycosis • Mycobacterial infections • Tertiary syphilis and other treponemal infections • Major aphthous ulcers
Treatment • Management of underlying intestinal symptoms (nonsteroid
anti-inflammatory drugs, systemic corticosteroids) • Local, oral mucosal lesions: monthly intralesional cortico-
steroid injections in areas of ulceration until improvement is noted; treatment as needed
• Episodic burst of systemic corticosteroids in association with local management of oral lesions; if condition persists, 7 to 10 days of prednisone with rapid taper to zero, with monitoring
• Management of any associated malabsorption may be helpful. • Metronidazole, 5-aminosalicylic acid, ileal-release budesonide
Prognosis • Related to response of intestinal symptoms to treatment