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