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Systemic disorders and sydromes with H & N manifestation Jan Laco, M.D., Ph.D.

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Presentation on theme: "Systemic disorders and sydromes with H & N manifestation Jan Laco, M.D., Ph.D."— Presentation transcript:

1 Systemic disorders and sydromes with H & N manifestation Jan Laco, M.D., Ph.D.

2 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 !!!

3 Cardiovascular diseases Kawasaki disease = mucocutaneous LN syndrome –Japan –Children –Mucositis –cervical lymphadenopathy –coronary arteritis  aneurysm  myocardial infarction

4 Cardiovascular diseases antihypertensive drugs –nifedipine  gingival hyperplasia

5 Respiratory tract diseases Tuberculosis –rare complication of open lung TBC –painless ulcer on dorsum of tongue –cervical lymphadenopathy –Mi: caseating granulomas

6 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

7 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

8 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

9 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)

10 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

11 Endocrine disorders and pregnancy Pituitary hyperfunction of growth hormone –gigantism –acromegaly jaws (condylar growth) + hands + feet

12 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

13 Endocrine disorders and pregnancy Diabetes mellitus –relative or absolute deficiency of insulin –oral: candidosis acceleration of periodontal diseases dry mouth oral lichenoid reactions (drugs)

14 Endocrine disorders and pregnancy Pregnancy –gingivitis –pregnancy epulis formation –recurrent aphthae

15 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

16 Haematological disorders - anaemias mucosal and skin pallor + fatigue + breathlessness + tachycardia atrophic glossitis (B 12) - first sign atrophy of filiform papillae angular stomatitis candidosis

17 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

18 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

19 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

20 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

21 Cervical lymphadenopathy - infections Bacterial –teeth, tonsils, face –TBC –cat-scratch disease Viral –herpetic stomatitis –infectious mononucleosis –HIV infection Parasitic –toxoplasmosis

22 Cervical lymphadenopathy - neoplasms Primary –Hodgkin´s lymphoma –non-Hodgkin lymphomas Secondary –carcinomas –melanoma –leukaemias

23 Autoimmune diseases middle aged women antibodies in blood rheumatoid arthritis Sjögren´s syndrome systemic lupus erythematosus systemic sclerosis

24 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

25 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

26 AIDS – oral infections Fungal –trush or other forms of candidosis –70% patients Viral –HSV stomatitis –EBV – hairy leukoplakia Bacterial –gingivitis + periodontitis

27 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

28 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


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