Granulomatous disease Flashcards
Give differential diagnosis of granulomatous diseases
Infection:
Tb
Cat-scratch disease
Tertiary syphillis
Immune-mediated:
Sarcoidosis
Orofacial granulomatosis
Wegener granulomatosis
What are 2 acute sarcoidosis syndrome?
Lofgren syndrome - erythema nodosum, bilateral hilar lymphadenopathy, arthralgia
Heerfordt syndrome - Parotid enlargement, anterior uveitis, facial paralysis, fever
What are triggering factors of sarcoidosis?
- infection( EBV, HHV8, mycobacterium)
2. environmental factors (dust, pollen, silica, etc)
Diagnosis of Sarcoidosis?
Clinical features of lesions Radiographic presentation Lymphadenopathy Biopsy of lesions - Schaumann bodies, asteroid bodies (nonspecific) Bloods - elevated ACE (pulmonary involvement) - CBC - eosinophilia, anemia, leukopenia, thrombocytopenia - elevated ESR
Skin test - Kveim test - intradermal injection or sterile human sarcoid tissue
Treatment of sarcoidosis?
Resolve spontaneously
Active tx for - progressive dz, cardiac/neurologic involvement, hypercalcemia, disfiguring skin lesions, serious ocular lesions
- corticosteroids
- antiinflammatory (methotrexate, azathioprine)
- TNF alpha antagonist (thalidomide, pentoxyfilline)
What is orofacial granulomatosis?
Nonspecific granulomatous inflammation
- that is not assoc with foreign material, infection, allergy
Presentation of orofacial granulomatosis
Cheilitis granulomatosa (nontender persistent swelling of lips)
Melkersson-Rosenthal syndrome (fissured tongue, facial paralysis, swollen lips)
Hyperplastic folds of sulcus
Cobblestone buccal mucosa
Diff dx of orofacial granulomatosa
- Chronic granulomatous disease
- Chrons disease
- Sarcoidosis
- TB
- Oral foci of infection
- Foreign material
- Allergy
How do u disgnose orofacial granulomatosis?
Clinical
And by exclusion - if have those 2 characteristic clinical features, look for Chrons ds presentation, sarcoidosis (Elevated ACE, Hypercalcemia, skin lesions), TB, foreign material, allergy (difficult to obtain), oral infection
*in which all can produce similar presentation
Biopsy - lymphocyte infiltrates, noncaseating granulomatous inflammation, with or without multinucleated giant cells
Must have negative special stains
Negative AFB stain
Negative foreign material
Tx of orofacial granulomatosis
Resolve spontaneously Topical or intralesional steroids Topical tacrolimus RT Azathioprine Cyclosporine A TNF alpha antagonists (thalidomide) Surgical recontouring (high rcurrence)
What is Wegener granulomatosis
Its a necrotizing granulomatosis affecting the lungs, kidney and small vessels
It has 3 presentations
- Generalized Wegener granulomatosis (classic)
- upper and lower respi tract
- renal involvement - Limited Wegener granulomatosis
- upper and lower respi tract
- no involvement of renal - Superficial Wegener granulomatosis
- skin and mucosa
Cause of Wegener granulomatosis
Inhaled antigen from environment
Infectious agent
What are oral manifestations of Wegener granulomatosis
Strawberry gingivitis - hemorrhagic and friable gingiva
Oral ulceration
What is TB oral manifestation?
usually of secondary infection- Chronic mucosal ulceration Swellings Nonhealing socket Mucosal granularity Mandibular swelling + intrabony lesions WITH neck mass (swelling); lymphadenopathy (ddx: scc)
Histopath of TB
Cell-mediated hypefrsensitivity (type 4)
Produce caseating granuloma (with lymphocytic, epitheloid histiocytes, multinucleated giant cells)
