Worldwide distribution

<40 y/o F>M

Present in winter & spring

More common in white ppl

Disease in blacks more severe

Uknown

Improper degradation of antigenic material, Spread by person-to-person transmission or shared exposure to env agent; Mycobacterium' HHV-8 DNA8/8 pts (skin, lymph nodes, oral); hepatitis C, interferon treatment

Skin, lymph nodes, lungs, salivary gland (mucoceles, enlargment, and/or xerostomia may mimic Sjogren's), eyes, skin, bone, any organ

Cough, dyspnea, fever, malaise, lupus pernio (vilolaceous, indurated on face, erythema nodosum-red elevated nodules on legs)

Erythema nodosum

Bilateral hilar lymphadenopathy

Arthralgia

Heerford's syndrome

Parotid enlargement

Anterior uveitis (eye, lacrimal gland enlargmenet)

Fever

Clinical findings

Chest radiography

Histology: lesions, lymph nodes, accessory salivary glands (granulomatous inflammation w/out necrosis "noncaseasting", negative stains for microorganisms)

Elevated serum angiotensin converting enzyme (anemia, thrombocytopenia, leukopenia, elevated sed rate, eosinophilia)

Skin test-Kviem test (not used today)

Noncaseating granulomatous inflammation

Discrete granulomas, Langerhans giant cells (foreign body type) Schaumann bodies - laminated basophilic calcifications asteroid bodies - stellate inclusions

May involve salivary glands

Important to rule out other causes of granulmatous inflammation

Spontaneous remission in 60% w/in 2 yrs

20% managed w/ corticosteroids CNS, cardiac involvement

10-20% do not resolve or respond to corticosteroid therapy

4-10% die of complications (lung, heart or CNS)

Nonspecific granulomatous inflammation of oral soft tissues: lips, gingiva, etc.

If lips only involved, referred to as cheilitis granulomatosa (of Miescher)

Lip swelling & other facial tissues

Responded to topical steroids

Triad of:

Recurrent lip swelling

Relapsing facial nerve paralysis

Fissured tongue

Granulomatous inflammation (more common)

Lymphoedematous (rare)

Orofacial granulomatosis, cheilitis granulomatosis, Melkersson-Rosenthal syndrome

Mycobacterial infection TB

Chronic granulomatosis disease

Crohn's disease

Sarcoidosis

Env antigen

Oral foci of infection

Foreign body gingivitis

Diagnosis may be difficult

Steroid injections, topical, systemic

Antihistamines

Minocycline daily

Diet: avoid cinnamon and benzoate

Surgery: reduction cheiloplasty

Long history of recurrent catalase-positive bacterial and fungal infections often beginning in childhood

Cannot kill phagocytized catalase producing mo

Abnormal neutrophil nitroblue tetrazolium reduction test

75% x-linked recessive, 25% autosomal recessive

Rare 1/500,000

Manage infections

Variable

Freq 1/30,000

40 y/os

Male=female

Necrotizing granulomatous inflammation involving upper and lower respiratory tract, necrotizing glomerulonephritis, systemic vasculitis anywhere in body

Chronic disease

Pseudoepithelilomatous hyperplasia

Subepithelial abscess

Extravasation of erythrocytes

Chronic disease, induce remission

Corticosteroids

Cyclophosphadmide

Cyclosporine

Trimethoprim-sulfamethoxazole (Bactrim)

Methotrexate

Chronic disease, induce remission (75%)

Requires close follow-up

Relapse occurs in 30% in months-20 yrs

Follow c-ANCA

Palatal petechiae in pts w/ allergy

Oral mucosa itches

Neg. pressure ruptures small blood vessels

Non-blanching red petechiae or bruising develops

Resolves w/out sequelae

Release of IgE-mast cell complexes

Reactions may be localized (stomatitis) or systemic (life-threatening)

Agents: antibiotics esp penicillin and sulfa

Resembles lichen planus clinically, histologically & immunologically

Most common sites: posterior buccal mucosa or lateral tongue

May be bilateral or lateral

Type 4 hypersensitivity rxn cell mediated by T cells

Delayed CD4+ T helper cells bind to antigen, causing site to becom red and indurated, usually w/in 2 days

Cell toxicity: CD8+ cytotoxic T cells kill antigen-bearing cells

Resembles pemphigus vulgaris clinically, histologically, and immunologically

Assocated with D-penicillamine, captopril and rifampin

Less sensitive than skin

1. Brief duration of contact

2. Saliva dilutes & neutralizes many antigens

3. mucosal structure facilitates rapid dispersal & absorption of antigens