Oral manif of system dis

Oral Manifestations of Systemic diseases

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A wide array of systemic diseases produce oral manifestations

• Congenital/developmental disorders• Nutritional disorders• Haematological diseases• Gastrointestinal diseases• Endocrine disorders • Dermatological disorders • Immunological disorders etc

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•Oral manifestation may herald the onset of some diseases or may be late manifestations•May be seen in oral soft tissues including the tongue•May occur in the jaw bones and/or the teeth•Both soft and hard tissue manifestations may

arise in some systemic diseases•A manifestation based approach is presented•Not comprehensive

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Angular cheilitis• Iron deficiency

anaemia• Folic Acid deficiency• Vit B12 deficiency• Vit B2 (riboflavin)

deficiency• Vit B6 (pyridoxine

deficiency)

• Malabsorption states (eg: Crohn’s disease)

• AIDS • Other immune defects

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A wide array of systemic diseases produce oral manifestations

• Congenital/developmental disorders• Nutritional disorders• Haematological diseases• Gastrointestinal diseases• Endocrine disorders • Dermatological disorders • Immunological disorders etc

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Glossitis including lingual mucosal atrophy

• Iron deficiency• Folate deficiency• Vit B12 deficiency• Vitamin B2

(riboflavin) deficiency• Vitamin B3(Niacin)

deficiency(pellagra)

• Vitamin B6 (pyridoxine) deficiency

• Tertiary syphilis

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Glossitis in deficiency states

in Iron deficiency Vitamin B complex

deficiency Vit B12 deficiency

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Enlarged tongue (macroglossia)

• Down’s syndrome• Hypothyroidism of infancy (Cretinism)• Mucopolysaccharidoses• Neurofibromatosis• Amyloidosis (multiple myeloma and others)• Acromegaly

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Fissured tongue

• Down’s syndrome• Sjogren’s syndrome and chronic xerostomic

states• Melkersson-Rosenthal syndrome• Multiple hamartoma and neoplasia

syndrome (Cowden’s syndrome)• Orofacial digital syndrome (sometimes cleft

tongue only)

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Macroglossia and fissured tongue in Down’s syndrome

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Tongue in severe xerostomia (Sjogren’s syndrome)

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Strawberry tongue

Hyperplastic fungiform papillae on a white or red background

• Scarlet fever• Mucocutaneous lymphnode syndrome

(Kawasaki disease)

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Erythema migrans

• Psoriasis• Reiter’s syndrome (may affect buccal

mucosa too)

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Erythema migrans

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White patches on oral mucosa• White sponge naevus• Dyskeratosis congenita• Pachyonychia congenita• Primary immuno-

deficiency diseases (Chronic Mucocutaneous Candidosis-CMC)

• Candidosis- endocrinopathy syndrome

• Chronic renal failure• AIDS and other

immunodeficiency states

• SLE• Pystomatitis vegetans• Syphilis, tertiary

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Some white lesions of systemic origin

White sponge naevus

Pachyonychia congenita Dyskeratosis

congenita

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Hairy leukoplakia in AIDS or other immunocompromised states

Pyostomatitis vegetans presenting as white patches

Eg. Ulcerative colitis

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CMC in Primary immunodeficiency disorders

eg. Congenital thymic aplasia (Di George syndrome)

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Burning sensation in the mouth

• Iron deficiency• Folate deficiency• Vitamin B12 deficiency• Vitamin B complex deficiency• Diabetes mellitus• Xerostomia of systemic causes• States of anxiety and depression• Hypothyroidism

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Numbness of orofacial areas (including lips)

• Intracranial neoplasia• Trotter’s syndrome• Leukaemia• Metastatic disease of the jaws• Diabetes mellitus• Sarcoidosis• Amyloidosis• Sickle cell disease

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Dry mouth

• Diabetes mellitus• Sjogren’s syndrome• Sarcoidosis • Ectodermal dysplasia• Drugs (anticholinergic, sympathomimetics, tricyclic

antidepressants)• Chronic renal failure• AIDS• Bone marrow transplantation (GVHD)

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Desquamative gingivitis

• Erosive lichen planus• Benign mucous membrane pemphigoid• Pemphigus• Dermatitis herpetiformis• Linear IgA disease

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Desquamative gingivitis

BMMP Pemphigus vulgaris

Linear IgA disease

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Swelling of lips

• Angioedema (allergic/ C1 esterase inhibitor deficiency)

• Crohn’s disease (orofacial granulomatosis)• Sarcoidosis• Ascher’s syndrome (double lip)

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Lip swellings of systemic origin

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Generalized gingival swellings

• Drug induced• Pregnancy• Leukaemia (AMML)• Aplastic anaemia• Hereditary Gingival

fibromatosis and its variants

• Amyloidosis• Mucopolysaccharidosis• A wide array of rare

syndromes

Some generalized gingival swellings

Acute myelomonocytic leukaemia

Hereditary gingival fibromatosis

Pregnancy gingivitisCyclosporin induced gingival

overgrowth

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Localized gingival swellings• Cowden’s syndrome• Sturge Weber angiomatosis• Focal epithelial hyperplasia (Heck’s disease)• Wegener’s granulomatosis (Strawberry gingivitis)• Langerhan’s cell tumours• Sarcoidosis• Crohn’s disease• Tuberous sclerosis

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Some localised gingival swellings of systemic origin

Strawberry gingivitis in Wegener’s granulomatosis

Gingival swellings in Tuberous sclerosis (mental handicap, epilepsy and sebaceous adenomas on skin)

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Spontaneous Gingival bleeding

• Idiopathic thrombocytopenic purpura• Leukaemia, Acute lymphoblastic leukaemia• Lymphoproliferative disorders• Myeloproliferative disorders• Myelodysplastic disorders• Scurvy• Ehlers-Danlos syndrome• Haemophilia, von Willebrandt’s disease

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Bleeding gingivae in leukaemia

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Blood blisters in the mouth

• Any cause of purpura• Mucous membrane pemphigoid• Amyloidosis• (Localized oral purpura- angina bullosa

haemorrhagica)

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Blood blisters

Amyloidosis Localized oral purpura- angina bullosa haemorrhagica

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Conical teeth

• Ectodermal dysplasia• Ellis- van Creveld syndrome

(Chondroectodermal dysplasia)• Incontinentia pigmenti (Bloch-Sulzberger

disease)

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Hypodontia

About 37 syndromes including • Ectodermal dysplasia • Down’s syndrome• Crouzon syndrome• Chondroectodermal dysplasia• Sturge Weber• Incontinentia pigmenti• Goldenhar

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Conical teeth and hypodontia in ectodermal dysplasia

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Delayed eruption of teeth

• Rickets• Hypothyroidism of infancy (Cretinism)• Hypoparathyroidism in early childhood• Hypopituitarism (Pituitary dwarfism)• Cleidocranial dysplasia• Biliary atresia (also green teeth)• Hereditary gingival fibromatosis and associated

syndromes

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Enamel hypoplasia of deciduous teeth

Deciduous dentition:• Intrauterine infection e.g. rubella• Intrauterine metabolic disturbance• Congenital hypoparathyroidism• Pseudohypoparathyroidism (hereditary)

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Enamel hypoplasia of permanent teeth (chronological)

• Severe rickets (disputed)• Childhood fevers• Coeliac disease • Cystic fibrosis• Childhood hypoparathyroidism• Biliary atresia• Gastroenteritis• Radiotherapy• Chemotherapy

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Enamel hypoplasia due to systemic disease

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Supernumerary teeth

About 18 syndromes including• Cleidocranial dysplasia• Gardner’s syndrome• Apert syndrome

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Mandibular prognathism

• Acromegaly• Down’s syndrome• Cleidocranial dysplasia• Gorlin Goltz syndrome• Crouzon syndrome• Apert syndrome

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Bad taste (non-oral causes)• Dry mouth of systemic

causes• Depression and anxiety states• Drugs (Metronidazole, ACE

inhibitors etc)• Nasal/pharyngeal disease

(Chronic sinusitis etc)• Diabetes• Pharyngeal pouch

• Gastric regurgitation• Liver disease• Uraemia• Temporal lobe

tumours • Temporal lobe

epilepsy• Bronchiectasis

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Dysphagia (non oral and oropharyngeal causes)

• Plummer Vinson syndrome

• Pharyngeal pouch• Oesophageal stricture• Oesophageal carcinoma• Scleroderma• Pressure from

mediastinal lymph nodes

• Achalasia of the cardia• Syringobulbia• CVA• Motor neurone disease• Guillain- Barré

syndrome• Myasthenia gravis• Muscular dystrophies

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Facial palsy• Bell’s palsy• CVA• Cerebral tumour• Parotid

tumour/malignancy• Diabetes mellitus• HIV infection• Cholesteatoma • Mastoiditis• Melkersson Rosenthal

syndrome• Sarcoidosis (Heerfordt

syndrome)

• Reiter’s syndrome• Multiple sclerosis• Ramsay Hunt

syndrome• Guillain- Barré

syndrome• Lyme disease• Leprosy• Kawasaki disease• Moebius syndrome

(congenital f.p)

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Facial swelling

• Allergic angioedema• C1 esterse inhibitor

deficiency• Systemic

corticosteroid therapy• Cushing’s syndrome• Myxoedema• Acromegaly• Nephrotic syndrome

• Superior vena cava syndrome

• Crohn’s disease• Sarcoidosis• Melkersson-Rosenthal

syndrome• Lymphoma

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Pigmentation• Drugs• Beta thalassaemia• Addison’s disease, Nelson’s syndrome• Biliary atresia• Peutz-Jeghers syndrome• Neurofibromatosis• Albright’s syndrome• Acanthosis nigricans• Laugier- Hunziker syndrome

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Melanotic macules on lip in Peutz Jeghers syndrome

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Ulcerations preceded by blisters

• Viral infections (Herpes simplex, Herpangina, Hand, Foot and mouth disease)

• Pemphigoid (usually BMMP)• Pemphigus vulgaris, Hailey- Hailey disease• Erythema multiforme• Epidermolysis bullosa• Dermatitis herpetiformis• Linear IgA disease

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Ulceration preceded by blisters

Pemphigus vulgaris Benign Mucous membrane pemphigoid

Epidermolysis bullosa

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Ulceration in syphilis

Chancre in primary syphilis

Mucous patches and snail-tract ulcers in secondary syphilis

Gumma in tertiary syphilis

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Ulceration of oral mucosa not preceded by blisters

• Haematinic deficiency • Behcet’s syndrome• Neutropenia (including

cyclic neutropenia)• Leukaemia • SLE• Crohn’s disease • Ulcerative colitis• Coeliac disease

• Drugs (e.g. methotrexate)• Reiter’s syndrome• Tuberculosis• Syphilis (all three stages)

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Ulceration in leukaemia

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Tuberculous ulcer

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Neuromas in the mouth

• Neurofibromatosis• Multiple Endocrine Neoplasia syndrome III

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Halitosis (of non oral/oropharyngeal origin)

• Drugs • Acute febrile illness• Respiratory tract

infections• Hepatic failure• Renal failure• Diabetic ketoacidosis

• Leukaemias• Sinusitis • Neoplasm in the

maxillary sinus• Volatile foodstuff

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Systemic diseases causing radiological abnormalities of the jaws

• Hyperparathyroidism and Hypoparathyroidism• Multiple myeloma• Haemoglobinopathies• Acromegaly• Osteogenesis imperfecta• Albers-Schonberg disease (marble bone dis)• Histiocytosis X• Fibrous dysplasia• Paget’s disease• Systemic sclerosis

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Oral manifestations of internal cancer

• Metastases in jaws or rarely oral soft tissues(Breast, Lung, Prostate, Thyroid, Kidney, Stomach)

• Effects of tumour metabolites– Facial flushing- carcinoid syndrome– Pigmentation (ectopic ACTH-secreting tumours)– Amyloidosis (multiple myeloma)– Oral erosions (glucaganoma)

contd…..

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Oral manifestations of internal cancer (contd..)

• Changes caused by other functional disturbances– Purpura, anaemia, infections (Leukaemia)– Infections (lymphoma, especially Hodgkin’s dis’se)– Bleeding (liver cancer)

• Anaemia (bleeding from gastrointestinal tumours)• Mucocutaneous diseases

– Acanthosis nigricans (gastric Ca)– Erythema multiforme (lymphoma, leukaemia )– Pempigus, pemphigoid (paraneoplastic pemphigus in

lymphomas) contd…..

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Oral manifestations of internal cancer (contd..)

• Inherited disorders with predisposition to internal malignancy and oral lesions– Gardner’s syndrome– Cowden’s syndrome– Multiple endocrine neoplasia syndrome type III– Neurofibromatosis– Gorlin Goltz syndrome– Tylosis– Tuberous sclerosis– Maffuci syndrome

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Conclusion• Oral manifestations of several systemic diseases are of

considerable diagnostic value • By recognizing them the dental clinician can contribute to

early diagnosis of the disease and thereby to the welfare of the patient.

• It is therefore imperative for dental surgeons to be aware of these manifestations

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