orofacial granulomatosis & crohn's disease, UC Flashcards
define orofacial granulomatosis (3)
- non-specific granulomatous inflammation
- non-caseating granulomas, multinucleate Langhan’s-type giant cells within the oral mucosa
- often considered a manifestation of Crohn’s, but may develop Crohn’s after OFG
OFG age group
adult (20-40yo)
common features of OFG (6)
- lip swelling/cheilitis granulomatosa
- cobblestoning (esp BM)
- mucosal tags (esp BM)
- cherry red gingival erythema and hyperplasia
- slit-like ulcers in sulci
- submandibular duct staghorning
management of OFG (6)
- diet restriction - cinnamon, benzoates, avoid preservatives
- topical calcineurin inhibitor or steroids
- intralesional triamcinolone at LM/lip boundary
- immunosuppression (pred, aza, mercaptopurine, methotrexate)
- immunomodulators (eg anti TNF)
- surgery - excise fibrous bands, cheiloplasty
what infection may arise from using topical corticosteroids for OFG?
pseudomembraneous candidiasis
possible side effect of intralesional triamcinolone at lip/LM boundary
hypopigmentation
what conditions are associated with OFG?
- Meischer’s syndrome (cheilitis granulomatosa)
- Melkersson-Rosenthal syndrome (labial and perioral swelling, LMN CVII paralysis, plicated tongue)
- foodstuffs type IV hypersensitivity
- sarcoidosis
- angioedema
what substances trigger a foodstuffs type IV sensitivity which may appear as OFG? (3)
- cinnamon
- cinnamaldehyde
- benzoate
give some of the features of Melkersson-Rosenthal syndrome (3)
- labial and perioral swelling
- LMN CVII paralysis
- plicated tongue
describe sarcoidosis
- systemic granulomatous condition (non-caseating)
- unknown aetiology with genetic predisposition
- middle-aged females, Afrocaribbean, Scandinavian
- most have spontaneous resolution but may have relapse/remission
sarcoidosis demographic
- middle-aged females
- Afrocaribbean and Scandinavian especially
list some of the IO (3) and EO (5) features of sarcoidosis
IO:
- loose teeth, bone pain
- swollen glands (parotid esp) +/- xerostomia
- soft tissue swelling, ulcers, gingivitis, pain (OFG-type symptoms)
EO:
- increased serum ACE and CXR hilar adenopathy
- dry cough, dyspnoea, chest pain
- skin bumps/raised broad lesions
- fever, malaise, weight loss, arthralgia
- dry eyes
what autoimmune disease does sarcoidosis mimic?
sjogren’s syndrome
describe Crohn’s disease
- inflammatory bowel disease causing chronic inflammation of GI tract
- young adult females (2nd peak in old age)
- aetiology largely unknown
- flares and asymptomatic intervals
GI s/s of Crohn’s disease (7)
- skip lesions and string sign, thickened bowel
- fissures/rosethorning, deep ulcers, fistulae
- abdominal pain, diarrhoea, abdominal distention
- anaemia, weight loss
- rectal bleeding, perianal sepsis
- transmural chronic granulomatous inflammation
- cobblestoning, excess mucus