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 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 • 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 • 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

Systemic disorders and sydromes with H & N manifestation