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

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 !!!

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

Cardiovascular diseases antihypertensive drugs nifedipine  gingival hyperplasia

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

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

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

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

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)

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

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

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

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

Endocrine disorders and pregnancy gingivitis pregnancy epulis formation recurrent aphthae

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

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

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

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

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

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

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

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

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

Autoimmune diseases Systemic lupus erythematosus Systemic sclerosis 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

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

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

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

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