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Systemic disorders and sydromes with H & N manifestation
Jan Laco, M.D., Ph.D.
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Cardiovascular diseases
• Infective endocarditis– source: bacteraemia after tooth brushing or
extraction– < 15% IE– valve defects: congenital x rheumatic fever– prosthetic valves– colonisation of cardiac valves vegetations
valve destruction
– !!! ATB cover !!!
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Cardiovascular diseases
• Kawasaki disease= mucocutaneous LN syndrome– Japan– Children– Mucositis– cervical lymphadenopathy– coronary arteritis aneurysm myocardial
infarction
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Cardiovascular diseases
• antihypertensive drugs– nifedipine
gingival hyperplasia
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Respiratory tract diseases
• Tuberculosis– rare complication of open lung TBC– painless ulcer on dorsum of tongue– cervical lymphadenopathy– Mi: caseating granulomas
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Respiratory tract diseases
• Sarcoidosis– chronic granulomatous disease of unknown
origin– lungs, LN (hilar), salivary glands– oral: painless swelling – gingivae, lips– diagnosis: biopsy of labial glands– Mi: non-caseating granulomas + calcifications– Heerfordt´s syndrome: parotis with
sarcoidosis + xerostomia + uveitis + fever
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Lethal midline granuloma syndrome
• clinically: destruction of central facial tissue + fatal outcome
• Wegener´s granulomatosis– systemic necrotising vasculitis (ANCA+)– granulomas of upper and lower RT– glomerulonephritis
• Angiocentric T cell lymphoma
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Gastrointestinal diseases
• Crohn´s disease– inflammatory disease of unknown origin– ileocaecal region - thickening and ulceration– oral: lips swelling
cobble stone thickening of buccal mucosa with painful small ulcers
– Mi: small granulomas
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Gastrointestinal diseases
• Gardner´s syndrome (AD inheritance)= multiple jaw osteomas + polyposis coli with malignant potential– dental defects, epidermal cysts
• Peutz-Jeghers syndrome– pigmented macules around lips + intestinal
polyposis (small bowel)
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Nutritional deficiencies
• vitamin A – squamous metaplasia keratinisation (leukoplakias ?)
• vitamin B2 – angular stomatitis - painful red fissures at angles
• vitamin B12 - pernicious anaemia– glossitis
• vitamin C - scurvy – gingival swelling and bleeding
• vitamin D - rickets
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Endocrine disorders and pregnancy
• Pituitary hyperfunction of growth hormone– gigantism– acromegaly
jaws (condylar growth) + hands + feet
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Endocrine disorders and pregnancy
• Adrenocortical diseases– Addison´s disease = cortical insufficiency – failure of cortisol and aldosteron secretion– early sign – oral pigmentations – gingivae, buccal
mucosa, lips
– Adrenal medulla– MEN IIb= phaeochromocytoma + hyperparathyroidism + thyroid
medullary carcinoma + oral mucosal neurofibromas
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Endocrine disorders and pregnancy
• Diabetes mellitus– relative or absolute deficiency of insulin– oral: candidosis
acceleration of periodontal diseases
dry mouth
oral lichenoid reactions (drugs)
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Endocrine disorders and pregnancy
• Pregnancy– gingivitis– pregnancy epulis formation– recurrent aphthae
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Haematological disorders - anaemias
• iron deficiency (microcytic a.)– chronic menstrual blood loss– chronic bleeding from peptic ulcer
• pernicious anaemia (macrocytic a.)– middle aged women– autoimmune chronic gastritis vitamin B12
deficiency+ neurological disease: degeneration of spinal
cord
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Haematological disorders - anaemias
• mucosal and skin pallor + fatigue + breathlessness + tachycardia
• atrophic glossitis (B 12) - first sign
• atrophy of filiform papillae
• angular stomatitis
• candidosis
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Haematological disorders - leukaemias
• = neoplastic disorder of bone marrow• acute x chronic• lymphoblastic x myeloblastic• ALL - children• AML - adults• anaemia + infection + bleeding tendency• hepatosplenomegaly + lymphadenopathy• oral: gingival swelling + mucosal ulcerations +
purpura
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Haematological disorders - haemorrhagic diseases
• bleeding after tooth extraction > 1 day
• 1. vascular disorder
• 2. platelet disorders – purpura, petechiae, ecchymoses– imm. follow trauma spontaneous stop
• 3. clotting disorders – long severe bleeding after short delay
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Haematological disorders - haemorrhagic diseases
• Idiopathic thrombocytopenic purpura– antibodies x platelets low number in blood– children, young women
• von Willebrand´s disease (AD inheritance)– low level of vW factor (part of FVIII)
• drugs associated– aspirin
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Haematological disorders - haemorrhagic diseases
• Haemophilia A (X inheritance)– most common– FVIII deficiency– childhood – bleeding into muscles or joints (haemarthros)
• Acquired disorders– liver diseases – vitamin K deficiency – anticoagulant treatment - warfarin
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Cervical lymphadenopathy- infections
• Bacterial– teeth, tonsils, face– TBC– cat-scratch disease
• Viral– herpetic stomatitis– infectious mononucleosis– HIV infection
• Parasitic– toxoplasmosis
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Cervical lymphadenopathy- neoplasms
• Primary– Hodgkin´s lymphoma– non-Hodgkin lymphomas
• Secondary– carcinomas– melanoma– leukaemias
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Autoimmune diseases
• middle aged women
• antibodies in blood
• rheumatoid arthritis
• Sjögren´s syndrome
• systemic lupus erythematosus
• systemic sclerosis
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Autoimmune diseases
• Systemic lupus erythematosus– antinuclear factors– 20% patients have oral symptoms– skin rash (butterfly) + arthritis + pleuritis +
glomerulonephritis– oral: lichenoid lesions
• Systemic sclerosis– subcutaneous and visceral fibrosis (GIT, lungs, …)– mask-like face + limited oral opening
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AIDS
• retrovirus HIV 1• sexual + i.v. drugs + blood transmission• deterioration of cell immunity (CD 4 T cell)• opportunistic infections• 75% patients have orofacial diseases
– infections– tumors– cervical lymphadenopathy
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AIDS – oral infections
• Fungal – trush or other forms of candidosis– 70% patients
• Viral– HSV stomatitis– EBV – hairy leukoplakia
• Bacterial– gingivitis + periodontitis
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AIDS – hairy leukoplakia
• almost unique to AIDS !!!• EBV virus• lateral borders of tongue• soft white painless area with corrugated surface• Mi: hyperkeratosis + koilocytes• No premalignant lesion• indicator of low immune status poor prognosis• may regress spontaneously
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AIDS - tumors
• 50% patients• Kaposi´s sarcoma – HSV 8
– purplish bleeding area or nodule on palate– Mi: vascular tumor - minute proliferating blood
vessels
• Non-Hodgkin lymphomas– painless swelling of palate + gingiva– Mi: high grade B cell lymphomas