Oral

manifestations

diseases

of

systemic

Presented by - Dr. Priyanka Airen

Contents
Introduction
Cardiac diseases
Hematological diseases
Liver diseases
Immunological diseases
Respiratory diseases
Rheumatological diseases
Oncological
Endocrine diseases
Renal diseases
Gastro intestinal
Eating disorders
Nervous disorders
Cutaneous disorders
HIV diseases
Drug induced
Infections
Multisystem

Conclusion

Introduction
Nature is nowhere accustomed more openly to display her secret mysteries thus in cases
where she shows traces of her workings apart from the beaten path: nor is there any
better way to advance the proper practice of medicine than to give our minds to the
discovery of the usual law of nature by careful investigation of cases of rarer forms of
disease for it has been found in almost all things, that what they contain of useful or
applicable nature is hardly perceived useless we are deprived of them, or they become
deranged in some way.

- William Harvey 1657

Correct treatment begins with a correct diagnosis. Arriving at a correct diagnosis is one of
the toughest jobs that we face as dentists. Not only does it require sound knowledge of
the diseases and their symptoms but also it is a skill and art to be mastered.
Diagnosis in dentistry may be defined as the process whereby the data obtained
from questioning by the dentist to identify deviations from the normal (Robinson 1963).
Correct diagnosis is the foundation for rational therapy and is thus the pioneer in
adequate treatment.
Milliard (1963) has noted that an accurate and complete diagnosis can only be the
result of a thorough and systematic collections of all available information about a
patient, and the logical arrangement and subsequent study of this information.
Thus although no single investigation establishes a diagnosis, the omission of a
relevant investigation may make correct diagnosis impossible.
Many systemic diseases have oral manifestations. The oral cavity might well be thought
of as the window to the body because oral manifestations accompany many systemic
diseases. These oral manifestations must be properly recognized if the patient is to
receive appropriate diagnosis and referral for treatment.. The lesions of the oral mucosa,
tongue, gingiva, dentition, periodontium, salivary glands, facial skeleton, extraoral skin
and other related structures caused by some of the more common systemic diseases are
highlighted here..

Systemic diseases often present with abnormalities of the structures of the mouth and
0jaws.
Proper diagnosis is essential to initiate the correct treatment. The primary care physician
and dentist must be familiar with these problems if their patients are to be well served.

CARDIAC DISEASES
Cardiac Diseases can be congenital or acquired. In children they are almost
always congenital, while the acquired are, rheumatic heart disease, hypertension and
atherosclerosis, and are very rare.
Congenital heart disease (CHD)
The etiology is in most cases unknown, but genetic factors, teratogenic agents
(viruses, drugs and alcohol) may be causal. CHD is prevalent in many of the syndromes.

Heritable disorders,

Chromosomal disorders (Trisomy 21)

Inborn errors of metabolism (Homocystinuria)

Connective tissue disorders (Martin Syndrome, Hurler syndrome, Osteogenesis

imperfecta).
The CHD is classified into cyanotic and non-cyanotic. In cyanotic type, blood is

shunted from right to left within heart due to a cardiac defect. The most common is
tetralogy of fallot. Other cyanotic failures are transposition of great vessels, pulmonary
stenosis with patent foremen ovale and pulmonary atresia.
In non-cyanotic type, there is no shunting from left to right in the heart occurs.
The most common form is ventricular septal defect (VSD), Aortic valve stenosis, atrial
septal defect (ASD). Patent ductus arteriosus (PDA), pulmonary valve stenosis and
coarctation of aorta are other non-cyanotic heart diseases.
Most patients with cyanotic heart diseases, irrespective of type and category,
share certain clinical characteristics and oral manifestations. The cyanotic category
generally exceeds the non-cyanotic category in security and manifestations. Clinical
characteristics include

exercise intolerance

Fatigability, lower respiratory tract infections, retarded physical growth, and delayed
puberty, Psychologic mal-adjustment and low tolerance to stress.

Oral manifestations: include cyanotic gingivitis, and stomatitis, and glossitis.

Characteristics of Teeth:
Delayed eruption of teeth, increased caries activity, increased structural defects of
enamel and intrinsic dychromia of the dentitions resulting from medications and/or blood
by-product.
Deposition:
The teeth have a paper-white appearance against a background of cyanotic
mucosa, lips and tongue. This tooth- color is related to mineralization disturbances.

Hematological diseasesERYTHORBLASTOSIS FETALIS- Erythroblastosis fetalis is a term used to describe the

hemolytic anemia of newborns caused by a blood incompatibility between the mother and
fetus. The immunological basis of the disease was recognized following red cell
agglutination studies (Levine et al., 1941). The dental findings include developmental
enamel the defects of varying degrees.

Clinical Manifestations
Erythroblastosis fetalis results from hemolysis of fetal red cells due to a maternalfetal antibody-antigen reaction. Since the development of anti-(D) immunoglobulin in the
mid-1960s, Erythroblastosis fetalis generally has been prevented (Beal 1979; Dornan
1982). However, other types of blood antibodies, called irregular antibodies, cause the
same destruction of red cells as the most potent irregular antibody, although it is
relatively rare.
The pathogenesis of anti-kell Erythroblastosis fetalis is identical to that caused by
anti-Rh (D). In others, the maternal antibodies coat the fetal red cells and cause
hemolysis. The fetus develops anemia with a resultant increase in the bilirubin content of
the amniotic fluid. The fetus compensates with extra-medullary erythropoiesis,
particularly by the liver spleen and kidneys (Bowman 1984). Hence, the term
Erythroblastosis fetalis is used to described this condition.
The newborn with severe disease appears pale and anemic, and presents with
hepatosplenomegaly, edema, and ascites. Shortly after birth, jaundice occurs as a result of
the high bilirubin levels. Based on this varied clinical picture, erythroblastosis fetalis also
is known as inters gravis neonatorum, anemia of the newborn, generalized edema of
the newborn, hydrous fetalis, and hemolytic disease of the newborn (Phibbs 1987).
In the past, there was a high correlation of neurologic damage in children with
Erythroblastosis fetalis. The primary cause was the neurotoxic effects of untreated
jaundice, referred to as kernicterus.
These children often have cerebral palsy, with or without mental retardation.
Currently, aggressive medical treatment to control bilirubin levels in the newborn has
reduced the extent of the bilirubin encephalopathy (Klemperer 1984).

Enamel defects may include hypoplasia limited to the primary dentition or

may affect the cusps of the permanent first molars (Gibson and Conchie 1964). The
extent of the dental disturbance coincides with the timing of the metabolic disturbance
(Gibson and Conchie 1964). Approximately 15% of the children present with teeth that
are discolored due to the incorporation of biliverdin, the by-product of bilirubin, which
causes jaundice (Forester and Miller 1955; Bevies 1956). The discoloration may be mild,
moderate, or severe, and are not interdependent (Watson 1955; Perlstein and Massler
1956).
The dental findings are a striking example of prenatal dental dysplasia associated
with Erythroblastosis fetalis (Forester and Miller 1955).
The site of the developmental defects of enamel corresponds to the time in-utero
when the maternal antibody titre was rising (26-28 th weeks in-utero), and the premature
delivery (Kraus and Jordan 1965). The defects are present from the incisal edges to the
neonatal line of Rushton (Rushton 1933). The neonatal line represents the more
pronounced lines of Reitzus where the ameloblasts were disturbed; thus the enamel
formation is thin or incomplete (Stein 1947). The more normal enamel has formed in the
post transfusion period (Mc Millan and Kashgarian 1961). The ring of hypoplasia
present on the maxillary canines has been referred to as the Rh hump, since it is
present in that from of erythroblastosis fetalis (Watson 1955). Also we can see a complete
ring of hypoplasia, since the clinical effect of the hemolytic disease is indistinguishable
from the cause.
The discoloration of his teeth was limited to yellow and was only noted on his
incisors, and perhaps was due to the thickening of the enamel rather than incorporation of
the by-products of the jaundice.
The ring of hypoplasia on the maxillary primary canine is characteristic of
Erythroblastosis fetalis.
Anemia
Irondeficiencyanemiaisthemostcommonhematologicaldisorder

Oralmanifestationsare
Atrophicglossitis,mucosalpallor,andangularcheilitis.Atrophicglossitis,flatteningof
thetonguepapillae,resultinginasmoothanderythematoustonguemaymimicmigratory
glossitis. Migratory Glossitis, also known as geographic tongue, is a condition of
unknownetiologythataffects1B2%ofThepopulation.Itresultsinlesionsonthetongue
that are erythematous, nonindurate, atrophic, and Bordered by a slightly elevated,
distinctrimthatvariesincolorfromgraytowhite.InatrophicGlossitis,theseareasdo
nothaveawhitekeratoticborderandtheyincreaseinsizeratherthanChangingposition.
In more severe cases, the tongue may be tender. Angular cheilitis, occurring at The
commissuresofthelips,iscausedbyCandidaalbicansinfectionresultinginreddening
and cracking, and some level of discomfort. dysphagia due to pharyngoesophageal
ulcerations.
Oral complications seen with sickle cell anemia include mandibular salmonella
osteomyelitis which results in areas of osteoporosis and erosion followed by
osteosclerosis. Anesthesia or paresthesia of the mandibular nerve, and asymptomatic
pulpalnecrosismayalsooccur.
These conditions are exacerbated when marrow proliferation is intense. Associated
dentofacialdeformitiesarecharacterizedradiographicallybyareasofdecreaseddensities
andcoarsetrabecularpatternmosteasilyseenbetweentherootapicesoftheteethandthe
inferiorborderofthemandible.Osteosclerosis.mayfollowthrombosisandinfarction.

Angular chelitis

Palatal bruising in
aplastic anaemia

Leukemia

Oral complications of leukemia frequently include gingival hypertrophy, petechiae,

ecchymosis,mucosalulcers,andhemorrhage.Lessfrequently,mentalnerveneuropathy,
called"numbchinsyndrome,maybethepresentingcomplaint.Palatalulcerationsand
necrosismayheraldthepresenceofmucormycosisofthenasalcavityandtheparanasal
sinuses.Sixteenpercentand8%ofchildrenwithacuteleukemiaarereportedtohave
gingivitisandmucositisrespectively.Bacterialinfectionsoftheoralcavity,whichmay
beasourceofsepticemia,arealsocommoninthesepatientsandmustberecognized
earlyandtreatedaggressively.
Treatmentofleukemia
chemotherapeuticagentscanresultinreactivationofherpessimplexvirus(HSV)leading
tooralmucositis.oral mucositiscanalsooccurfromchemotherapywithoutanHSV
component, since thinning of the surface layer of mucosa and/or bone marrow
suppressionallowsforopportunisticorganismstoinvadethemucosa.

Gingival
enlargement

Leukemicinfilterate

Gingival
bleeding

Multiplemyeloma

When multiple myeloma involves the oral cavity, it is usually a late secondary
manifestationoflesionswithinthejaws,mostoftenthemandible.Theselesionscause

swelling of the jaws, pain, numbness, mobility of teeth, and pathologic fractures
.Punchedoutlesionsoftheskullandjawarecharacteristicradiographicfindings.The
incidenceofjawinvolvementinmultiplemyelomaaveragesabout15%).Sincemultiple
myelomaresultsinimmunosuppression,avarietyofinfectionsmaybepresent,including
oral hairy leukoplakia and candidiasis Amyloi.d deposits in the tongue can lead to
macroglossia.

LIVER DISEASES
END-STAGE LIVER DISEASE AND LIVER TRANSPLANTATION
In children, chronic liver disease has a variety of causes including extra hepatic
biliary artesian, biliary hypoplasia, chronic active hepatitis, post-viral hepatitis and some
metabolic diseases such as tyrosinemia and - 1 a tyrosine deficiency.
The affected children present with neonatal jaundice and although early
intervention with porotoenterostomy may be successful, in failure cirrhosis and end
stage liver disease.
Nutrition is affected with general malabsorption of fats and fat soluble Vitamins
A,D,E, and K. Other complications include bleeding tendencies due to poor vitamin K
absorption and inadequate synthesis of clotting factors and prothrombin in the liver. In
addition, osteopenia and rachitic changes may be observed in the skeleton as a result of
impaired vitamin D metabolism.
Chronic liver disease in children may have many oral manifestations such as
green staining of the teeth and gingival as well enamel hypoplasia.
ENAMEL HYPOPLASIA
In every patient enamel hypoplasia was noted. On all erupted teeth, the enamel
defects ranged from minor breaks in the enamel to large areas of missing enamel, and
appeared to be mainly located in those areas of the primary teeth that were formed
postnuptially.

STAINING OF TEETH

Staining of the crowns of teeth was noted clinically in all the patients. The stains
ranged in color from yellowish-brown to deep green, with varying color intensity from
patient to patient.
Green or brown staining of the teeth and gingival tissues by bilirubin pigments is
the result of hyperbilirubinemia associated with chronic liver failure. Enamel hypoplasia,
often manifested as areas of missing enamel is another common finding. These enamel
defects may have been caused by changes in the organic matrix of developing enamel
resulting from metabolic disturbances, but are more likely to have resulted from the
effects of osteopenia and other disturbances of calcium and phosphate metabolism
encountered in chronic liver disease. Similarly, an enlarged pulpal chamber, which is a
manifestation of dentin hypoplasia, is the likely result of hepatic rickets. Delayed dental
eruption most probably reflects the general retardation of growth and development
observed in chronic liver disease.
HYPERBILIRUBINEMIA
The presence of hyperbilirubinemia during enamel and dentin formation results in
dental pigmentation, due to accumulation of intrinsic staging by bilirubin oxidation,
Bilirubin is a breakdown product from red blood cells, which, at high levels can cause
jaundice and accumulate in the interstitial fluid, mucosa, and skin, resulting in
discoloration that can vary from yellow to deep shades of green.
Intrinsic pigmentation occurs during dental formation and has been frequently
related to drug administration- particularly tetracycline-and systemic alterations present
at birth, such as hemolytic anemia in newborns.

In newborns, other complications may be associated with discoloration of teeth,

such as biliary artesian, premature birth, neonatal respiratory dysfunction, significant
internal hemorrhage, respiratory dysfunction, congenital hypothyroidism, biliary
hypoplasia, meta- bolic diseases, hepatic dysfunction and generalized hyperbilirubinemia.
Herbert and Declaimer reported the presence of iatrogenic anemia, direct and indirect
hyperbilirubinemia, and neonatal staphylococcal sepsis, among other findings, in cases of
green dental pigmentation.
Barter et al., described primary teeth pigmentation due to blood incompatibility
and verified that dental discoloration became less evident at the third year of life.
The extent of discoloration and its intensity were directly related to the duration
and severity of the pathology, respectively in most cases occurring only in primary
dentition.
Green pigmentation may be seen through the translucent enamel and, presumably,
is the result of a reaction of the developing tooth to the blood supply, which is rich in
bilirubin serum. The degree of pigment deposition may be proportional to the serum
concentration of bilirubin and may disappear gradually.
The calcification process of primary teeth begins during the fourth month of fetal
life and ends almost at the 11th month after birth on the primary second molars. At birth, a
significant portion of upper and lower central and lateral incisors exhibits more than half
of the already formed crown.
The crowns of the first molars begin to form at birth, and the hypotheses of
permanent dentition being affected by icterus generated by Erythroblastosis fetalis is very
unlikely, except for these teeth. Morisaki et al., agree with these conclusions, having
found green pigmentation possibility of the permanent first molars should be considered,
mainly in view of the stain localization of deciduous teeth and high bilirubin levels.

Respiratory disorders have been reported as a cause of neonatal incisors without

interruption of the mechanism of keeping the ductus arterioles functionally patent. This
cause poor perfusion of the hepatic sinusoids and decreasing bilirubin excretion. Both
cases show similarities in respect to respiratory disorders, anemia, icterus, and high
bilirubin levels exhibited in the neonatal period.

IMMUNOLOGICAL DISORDERS
DECREASES IN T- CELL NUMBERS (DI GEORGE SYNDROME)
After production in the bone marrow, progenitors of T lymphocytes migrate to the
thymus for further selection and differentiation. In Di George syndrome, the thymus can
be partially or completely absent due to an abnormal migration of the third and fourth
bronchial pouches consequently.

AGAMMAGLOBULIN

T-cells fail to mature if there is no thymus, which severely decreases the T-cell
population. The immunodeficiencies found in this patient group can range from slight to
severe.
Infectious complications of Di George syndrome include oral candidasis, herpetic
infections, pneumonia, chronic rhinits and susceptibility to all viral and opportunistic
infections such as Pneumocystis carinii. In addition, other cardiac and craniofacial
defects, including cleft palate, hypertelorism and malformed ears develop in this
syndrome from disturbances in embryological development. In some children, the
parathyroids do not develop which may contribute to enamel hypoplasia. After a common
gene was identified in these patients, the name DiGeorge / Velo-cardio-facial syndrome
wassuggested.

RESPIRATORY DISEASES
INFANTILE SYSTEMIC HYALINOSIS
Infantile systemic hyalinosis is a rare fatal condition in which the symptoms appear
soon after birth and death usually occurs before two years of age.
It is probably an autosomal recessive condition. The systemic features are essentially
due to widespread deposition of hyaline material in tissues. These include thickening and
nodularity of skin, growth failure, joint contractures, osteoporosis, diarrhoea and recurrent
infections.
Oral examination revealed an extensive overgrowth of gingival tissues with almost total
coverage of deciduous primary incisor teeth. The gingivae were soft, flabby and inflamed. The
rest of the oral mucosa felt thick on palpation but was of normal appearance.
The oral changes in a child with infantile systemic hyalinosis described here include
gingival fibromatosis, osteoporosis, altered root morphology. Sparsity of secondary cellular
cementum and replacement of the periodontal ligament by hyaline fibrous material.

Rheumatological Diseases
Sjogrens Syndrome
Sjogren's syndrome (SS) patients frequently present with xerostomia and parotid gland
enlargement. SS is often associated with rheumatoid arthritis. In one study 88% of the
patients with SS had abnormal submandibular/sublingual salivary flow, and 55% had abnormal
parotid flow. Enlargement of the parotid or submandibular gland is apparent in 35% of patients
with SS.
Xerostomia can be associated with fissured tongue, depapillation and redness of the tongue,
cheilitis,
and candidiasis .
Swallowing and speaking may become difficult due to persistent xerostomia. Bacterial parotitis,
which is usually accompanied by fever and purulent discharge from the gland, may also occur.

There is an increase in dental caries, especially around the cervical region of the teeth. It is
important to recognize SS quickly and refer the patient to a dentist since dental caries can
progress rapidly .Diagnosis is usually confirmed by labial minor salivary gland biopsy.
Histologically, there is a periductal lymphocytic infiltrate.

Scleroderma (Progressive Systemic Sclerosis)

Scleroderma is a chronic disease characterized by diffuse sclerosis of the skin, gastrointestinal
tract, heart muscle, lungs, and kidney .The lips of a patient with scleroderma may appear to be
pursed due to constriction of the mouth aperture, thus making it difficult to open the mouth.
Stomatognathic function, denoting the mouth and jaws collectively, is also difficult. Esophageal
fibrosis leads to lower esophageal sphincter hypotension and gastroesophageal reflux in 75% of
scleroderma patients .Symptoms include dysphagia and heartburn. The oral mucosa appears pale
and feels rigid. Multiple telangiectasias may be present.. The tongue can lose mobility
andbecome smooth in appearance as the palatal rugae flatten. Salivary hypofunction can also be

present

scleroderma

although usually to a lesser degree than in Sjogren's syndrome. Radiographically, the periodontal
ligament space is often thickened.
Lupus Erythematosus (LE)
Lupus erythematosus occurs as discoid lupus erythematosus (DLE) and systemic lupus
erythematosus (SLE]Oral lesions oc usually begin as an irregular whitish area that
extends peripherally . As they extend, the central area may become red and ulcerated while the
border remains elevated and hyperkeratotic. Oral lesions of lichen planus are similar to those of
DLE both clinically and histologically. Strict histologic criteria must be applied to distinguish
one from the other .
Oral or nasopharyngeal ulceration is recognized as a major diagnostic manifestation of SLE by
the American Rheumatism Association Committee on Diagnostic and Therapeutic Criteria. These
ulcerations are generally painless and often involve the palate. Purpuric lesions such as
ecchymoses and petechiae may also occur. In up to 30% of patients with SLE, salivary gland

involvement may occur concomitantly, leading to secondary Sjogren's syndrome and severe
xerostomia.

Rheumatoid Arthritis
The temporomandibular joint (TMJ) is often involved in rheumatoid arthritis. This is
usually characterized by erosions in the condyle leading to a decreased range of motion of the
mandible with pain upon movement. Oral dryness and salivary gland swelling can also be found
in patients with rheumatoid arthritis. These patients can also develop secondary Sjogren's
syndrome .Limited jaw function may necessitate TMJ reconstruction once the active disease is
controlled.
Prosthetic joints may provide solution for those patients severely affected with rheumatoid
arthritis.