Systemic disorders and sydromes with H N manifestation

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 B 2 – angular stomatitis - painful red fissures at angles • vitamin B 12 - 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 • 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 B 12 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 v. W 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 – 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 – 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