ORAL' MANIFESTATIONS IN SYSTEMIC DISEASES ...
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<strong>ORAL'</strong> <strong>MANIFESTATIONS</strong> <strong>IN</strong> <strong>SYSTEMIC</strong> <strong>DISEASES</strong>:<br />
HYPOVITAM<strong>IN</strong>OSES AND BLOOD DYSCRASIAS<br />
Charles Tomes Lecture delivered at the Royal College of Surgeons of England<br />
on<br />
6th July, 1951<br />
by<br />
H. H. Stones, M.D., M.D.S., F.D.S.R.C.S.,<br />
Professor of Dental Surgery, University of Liverpool.<br />
THERE IS A wide diversity in the oral manifestations of systemic diseases,<br />
and these depend both on the particular condition and sometimes on<br />
the age of onset. Thus in the hereditary disease osteogenesis imperfecta,<br />
the structure of the dentine may be affected, in which case the teeth vary<br />
in colour from yellow to bluish-black. Severe metabolic disturbances<br />
affect the developing and calcifying teeth. In icterus gravis, which is<br />
associated with the Rh factor, the deciduous teeth may be pigmented<br />
bluish-green. After several years the colour gradually becomes yellowishbrown.<br />
As the jaundice usually improves before calcification starts in<br />
the permanent dentition they are not usually involved. In various<br />
osteodystrophies such as generalized osteitis fibrosa, the jaw bones may<br />
be affected. Again, in certain diseases of the nervous system, there may<br />
be paralysis of the muscles of the palate and tongue or other muscles,<br />
or in others there may be neuralgia.<br />
Many systemic diseases have an effect on the oral mucosa.<br />
The following list (Stones, 1951) gives a classification of stomatitis and<br />
other non-neoplastic lesions of the oral mucosa on an aetiological basis,<br />
and it can be seen that the majority of them are associated with<br />
disturbances affecting other parts of the body.<br />
A. Traumatic, thermal, chemical, galvanic, actinic and irradiation<br />
lesions.<br />
B. Catarrhal stomatitis.<br />
C. Stomatitis due to hypovitaminoses.<br />
D. Stomatitis in the blood dyscrasias.<br />
E. Stomatitis associated with specific micro-organisms or larvae.<br />
F. Stomatitis associated with virus diseases.<br />
G. Stomatitis associated with allergic conditions.<br />
H. Stomatitis due to ingestion of metals and drugs.<br />
I. Other miscellaneous diseases chiefly of obscure origin with oral<br />
manifestations.<br />
Thus the oral manifestations in systemic disease present an extensive<br />
and fascinating study, and of these it is proposed to discuss the effect of<br />
the hypovitaminoses and the blood dyscrasias. Though observations<br />
will be chiefly directed to the oral changes, and these are important as<br />
they sometimes exhibit the first signs of a serious disease, it must be<br />
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ORAL <strong>MANIFESTATIONS</strong> <strong>IN</strong> <strong>SYSTEMIC</strong> <strong>DISEASES</strong><br />
emphasized that this is only one part of the organism and that a general<br />
examination and, frequently, laboratory investigations are necessary,<br />
before arriving at a diagnosis.<br />
THE ORAL <strong>MANIFESTATIONS</strong> OF HYPOVITAM<strong>IN</strong>OSES<br />
The chief vitamins concerned with the subject are the vitamin B<br />
complex and vitamin C. Deficiency of vitamins K and P are mentioned<br />
when considering purpura.<br />
Vitamin B Complex Deficiency<br />
A deficiency of some components of the vitamin B complex is known<br />
to have a marked effect on the oral tissues and this has been shown both<br />
from observations in man and animal feeding experiments. The effect<br />
of other components has not yet been worked out. There are often the<br />
clinical features of multiple deficiencies.<br />
The usual cause is a marked deficiency in the diet but the other factor<br />
is the capacity of the organism to maintain the vitamin in a stable form,<br />
and to assimilate it.<br />
Aneurin or thiamnin deficiency.-It has been known for some years that<br />
deficiency of this vitamin produces peripheral multiple neuritis as occurs<br />
in beri-beri. It is doubtful if there are any definite oral lesions, though<br />
Weisberger (1941) reported pinpoint herpetic-like vesicles occurring on<br />
the palate and undersurface of the tongue that were cured by the<br />
administration of aneurin. Mann, Spies and Springer (1941) state that<br />
it may cause hyperaesthesia of the oral mucosa.<br />
Riboflavin deficiency.-This produces characteristic oral lesions. They<br />
have been described by Sebrell and Butler (1939) in their riboflavin<br />
deficiency experiments on eighteen otherwise healthy women, and by<br />
various observers including Sydenstricker (1941), Jones et al. (1944),<br />
and by Braun, Bromberg and Brzezinski (1945) in their report on<br />
pregnant women in Jerusalem. An interesting contribution has recently<br />
been made by Welbourn, Hughes and Wells (1951) who have noted that<br />
following subtotal gastrectomy about 10 per cent. of cases develop the<br />
characteristic features of aneurin and riboflavin deficiency, either singly<br />
or combined. The most frequent is a hyporiboflavinosis, though without<br />
the desquamation of the nasolabial folds and ocular lesions. As both<br />
aneurin and riboflavin are only stable in acid solution it may be that with<br />
the achlorhydria following subtotal gastrectomy, a proportion of both is<br />
destroyed. They point out that Melnick, Robinson and Field (1941)<br />
have shown that up to 50 per cent. of thiamin may be destroyed by<br />
incubation with bile or pancreatic juice at a pH of 8-0 to 8 5, though<br />
the question of the stability of riboflavin has not been worked out in the<br />
same detail.<br />
In ariboflavinosis there is cheilosis in which the lips become red and<br />
cracked. A characteristic feature is the development of red painful<br />
areas at the angles of the mouth which in severe cases lead to the<br />
formation of several fissures resembling perleche. An important<br />
235
H. H. STONES<br />
predisposing factor is loss of the vertical dimension of the bite and<br />
consequent sagging of the tissues as occurs in elderly edentulous patients.<br />
Hence it is necessary to correct this loss (Ellenberg and Pollack, 1942;<br />
Mann, Mann and Spies, 1945; Mann, Dreizen and Spies, 1948).<br />
A painful glossitis is a feature in which there is sometimes engorgement<br />
of the fungiform papillae, the denuded summits of which permit the<br />
underlying capillary loops to appear as red elevations. It may be a<br />
magenta colour. Later there is atrophy of the papillae so that the<br />
tongue appears glazed, shiny and fissured.<br />
Other rarer features that have been reported are inflammation of the<br />
eyelids, conjunctivitis, disturbances of vision, and desquamation of the<br />
epithelium of the nasolabial folds, alae nasi, eyelids and ears.<br />
Nicotinic acid deficiency.-Pellagra is the chief disease associated with<br />
a deficiency and in this Spies, Bean and Asche (1939) point out that<br />
there is a multiple deficiency of the vitamin B complex including that of<br />
riboflavin and aneurin. Secondary pellagra, in which there is failure to<br />
assimilate the vitamin, has been described as occurring in association<br />
with chronic intestinal disturbances such as peptic ulcer, carcinoma of<br />
the stomach and ulcerative colitis (Bean, Spies and Blankenhorn, 1944).<br />
In the early stages the tip and margins of the tongue become red.<br />
This intensifies and the tongue becomes swollen and has a burning<br />
sensation. As the condition progresses the lingual papillae atrophy<br />
and there is desquamation of the superficial epithelial layers leaving a<br />
red, smooth and glazed surface (Kruse, 1942). Gingivitis or stomatitis<br />
which may be associated with fusospirochaetal organisms may develop,<br />
the inflammation starting at the interdental papillae and progressing<br />
until the gingivae are ulcerated. There is inflammatory involvement of<br />
other mucous membranes of the alimentary tract and sometimes of the<br />
perineum and vulva.<br />
Other features include dermatitis with the formation of small<br />
erythematous patches that eventually turn brown and desquamate. There<br />
may be mental symptoms of varying severity.<br />
The pellagrin often complains of tingling and numbness of the<br />
extremities which are characteristics of beri-beri which may co-exist with<br />
the disease.<br />
The administration of nicotinic acid produces a striking improvement<br />
in the inflammation of the mucous membranes, skin lesions and mental<br />
symptoms. It does not, however, cure the angular lesions of the mouth<br />
which arise from riboflavin deficiency, or the peripheral neuritis which is<br />
due to aneurin deficiency.<br />
Pyridoxine deficiency.-There is obscurity regarding the effects of<br />
pyridoxine deficiency in man and no definite conclusions have been<br />
reached. With the introduction of one of the antimentabolites desoxypyridoxine,<br />
limited tests have been carried out on its effect. Mueller<br />
and Vilter (1950) report eight cases who received 60 mgs. or 150 mgs. of<br />
desoxypyridoxine daily. The other components of the B complex were<br />
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ORAL <strong>MANIFESTATIONS</strong> <strong>IN</strong> <strong>SYSTEMIC</strong> <strong>DISEASES</strong><br />
at a low level but within normal range. After two to three weeks, oral<br />
lesions developed similar to a combined riboflavin and nicotinic acid<br />
deficiency. These remained unchanged when the latter vitamins, together<br />
with aneurin, were given, but they disappeared within 48-72 hours after<br />
pyridoxine was administered.<br />
Pantothenic acid deficiency.-The effect of a deficiency of pantothenic<br />
acid has not been elucidated in man, and the few reports in the literature<br />
are based on the therapeutic test. Field, Green and Wilkinson (1945)<br />
report six cases with glossitis and cheilosis at the angles of the mouth,<br />
which were relieved by calcium pantothenate after failure of other<br />
fractions of the vitamin B complex. The glossitis of these patients was<br />
characterized by atrophy of all the papillae, absence of coat, and by<br />
redness which was usually slightly purplish and darker than the scarlet<br />
red of nicotinic acid deficiency.<br />
Vitamin C Deficiency<br />
Scurvy, which results from extreme depletion of vitamin C, is now<br />
comparatively rarely seen in Great Britain. In four cases out of those<br />
that have been personally observed the gingival condition has led to a<br />
diagnosis of the deficiency. It typically occurs in middle aged or elderly<br />
bachelors who neglect their diet.<br />
There are marked oral lesions. The gingivae become swollen, maroon<br />
coloured and spongy, tending to cover the teeth. Haemorrhages<br />
frequently occur and the gingivae slough. Ecchymoses may also occur<br />
on the palate and inner aspect of the cheeks. The lips and skin may<br />
appear pale due to secondary anaemia. There is a tendency to bleeding<br />
from other sites; thus subperiosteal haemorrhages occur in the long<br />
bones so that bruising, particularly on the inner aspect of the knees and<br />
near the ankle, is frequently observed. Effusion of blood into joints<br />
causes haemarthroses.<br />
Infantile scurvy.-Barlow's disease is seen in infants during the first<br />
year, occurring in those who are bottle fed. Sometimes the first sign is a<br />
purplish swelling of the gingivae round several teeth; this has recently been<br />
observed in an infant, in whom a diagnosis of scurvy was.made from the<br />
gingival appearance. The limbs are tender due to subperiosteal<br />
haemorrhages, and the legs are characteristically flexed and everted;<br />
ossification is distorted, and the lesions are usually bilateral.<br />
It is doubtful, however, if a comparatively low intake has any marked<br />
effect on the incidence of gingivitis. Undoubtedly during the world<br />
wars in the winter months in Great Britain, there was a deficiency of<br />
ascorbic acid in the diet. In an investigation carried out by Stones,<br />
Lawton, Bransby and Hartley (1949) in an institutional school during<br />
the second world war, the vitamin C intake was estimated at only 20 per<br />
cent. of the normal requirements in the winter months, but was adequate<br />
in the summer months. Yet with over 250 children there were but few<br />
cases of gingivitis including only two of fusospirochaetal stomatitis<br />
237<br />
18
H. H. STONES<br />
observed during the three years commencing in 1942. Most observers<br />
have been unable to establish any correlation between low plasma levels<br />
of ascorbic acid and the more frequent type of gingivitis (Restarski and<br />
Pijoan, 1944).<br />
It may happen that a patient is suffering from a deficiency of more<br />
than one of the vitamin components and in this case the clinical features<br />
of the various deficiencies may occur together; as already mentioned<br />
this particularly happens in connection with the vitamin B complex and<br />
may also occur with vitamins B and C. Further, and particularly in<br />
nutritionally neglected old and middle aged cases, there may be an<br />
anaemia in which the blood picture shows a marked reduction both in<br />
the haemoglobin and red cell count.<br />
Hence these possibilities must be evaluated when instituting treatment<br />
and any suspected deficiency including that of iron must be covered.<br />
THE ORAL <strong>MANIFESTATIONS</strong> OF THE BLOOD DYSCRASIAS<br />
In the blood dyscrasias a variety of oral manifestations may be<br />
produced depending on the particular disease. As will be seen, in some<br />
conditions they are but slight and in others severe. It is very important<br />
for the dental practitioner to recognise the signs as he may be in a<br />
position to be the first to examine a patient with a serious or fatal<br />
disease, or sometimes if he operates without appreciating its significance,<br />
the patient may be subjected to a dangerous risk. It is proposed to<br />
mention representative types that illustrate the effect on the oral mucosa.<br />
The aetiology and haematology are reviewed by Sturgis (1948) and<br />
Whitby and Britton (1950).<br />
Hypochromic Anaemia<br />
Idiopathic hypochromic anaemia.-This usually occurs in young and<br />
adult women and is caused by a deficient absorption of iron, which is<br />
frequently associated with the hypochlorhydria that so often accompanies<br />
the condition.<br />
The blood picture usually shows that the red cell count is somewhat<br />
reduced and red blood corpuscles are smaller than normal. The colour<br />
index is low, being about 0 5 to 0 6 while the haemoglobin is reduced<br />
to 40 per cent. or 50 per cent.<br />
The oral mucosa is pale and there is a tendency to bleed from the<br />
gingivae. The tongue is occasionally smooth due to atrophy of the<br />
filiform papillae, and sometimes shows indentations from the teeth.<br />
The general symptoms include pallor, breathlessness and palpitation.<br />
There may be koilonychia, that is atrophic, thin and spoon-shaped nails.<br />
The condition improves following the oral administration of iron<br />
preparations.<br />
Plummer-Vinson Syndrome.-This condition usually occurs in middle<br />
aged females. It is associated with hypochromic anaemia and sometimes<br />
with hypochlorhydria. There may be an associated vitamin B complex<br />
deficiency.<br />
238
ORAL <strong>MANIFESTATIONS</strong> <strong>IN</strong> <strong>SYSTEMIC</strong> <strong>DISEASES</strong><br />
There are atrophic lesions of mucosal surfaces including the buccal<br />
and pharyngeal mucosa. Dysphagia is a feature. The tongue is smooth<br />
and sore, due to atrophy of the filiform papillae, and sometimes it is<br />
wrinkled. The mouth may show angular lesions as occurs in riboflavin<br />
deficiency.<br />
There is paleness, weakness and sometimes koilonychia. Ahlbom<br />
(1935) cotlsiders that this syndrome is responsible for a high proportion<br />
of the buccal and pharyngeal cancer that occurs in women. Darby (1946)<br />
emphasizes the importance of the iron deficiency and reports six<br />
representative cases in which the oral condition remained after vitamin B<br />
complex therapy but was alleviated with the administration of iron.<br />
Pernicious Anaemia<br />
This type of macrocytic anaemia has now been shown to be due to<br />
a deficiency of an anti-anaemia substance. The latter is normally<br />
provided through the interaction of the intrinsic factor of Castle which<br />
is in the gastric secretion, with the extrinsic factor which is thought to be<br />
a thermostable component of the vitamin B complex as yet unidentified.<br />
Achlorhydria is a most constant finding.<br />
Diagnosis is made from the blood picture. The red cell count is low,<br />
even falling below 1 million per c.mm., and there is anisocytosis,<br />
poikilocytosis and nucleated red cells. The colour index is high. There<br />
is a leukopenia.<br />
A characteristic early feature that occurs in about two-thirds of the<br />
cases is recurrent soreness of the tongue. During an exacerbation the<br />
tongue becomes very painful and red. The whole of the dorsum is<br />
usually affected though it may be limited to certain areas. Sometimes<br />
there is ulceration. In many cases, if untreated, there is eventually<br />
atrophy of the filiform papillae and desquamation of epithelium so that<br />
the tongue appears smooth-the typical Hunter's glossitis. It is nearly<br />
always clean.<br />
The lips and oral mucosa, including that of the palate, occasionally<br />
have a pale yellowish appearance.<br />
The skin sometimes has a pale yellowish tint. There are neurological<br />
and gastro-intestinal symptoms.<br />
With intramuscular injections of liver extract or vitamin B12 the oral<br />
and general symptoms are alleviated.<br />
Sprue<br />
In this disease, a macrocytic hyperchromic anaemia and multiple<br />
vitamin deficiencies may occur during its course. The cause is unknown<br />
and it is noteworthy that though it is endemic in certain tropical regions<br />
the natives are not usually affected, an exception to this being in Puerto<br />
Rico. In recent years non-tropical sprue or idiopathic steatorrhoea has<br />
been observed in more temperate climates.<br />
The outstanding pathological feature is that the mucosa of the<br />
239<br />
18-2
H. H. STONES<br />
alimentary tract from the oral cavity to anus tends to undergo atrophic<br />
changes, and the liver and pancreas may also atrophy. Hence<br />
steatorrhoea is a feature of the disease. The bone marrow may show<br />
aplastic or hyperplastic changes.<br />
In view of the mucosal changes a multiple vitamin deficiency is to be<br />
expected and Cayer, Ruffin and Perlzweig (1945) have shown that there<br />
are low values for certain components of the vitamin B complex,<br />
particularly thiamin and riboflavin, though the nicotinic acid level is not<br />
much affected, and for vitamins A and C.<br />
The oral lesions usually occur after the typical general symptoms.<br />
The most noteworthy change is in the tongue which may show atrophy<br />
of the papillae and desquamation of epithelium so that it becomes smooth,<br />
red and sensitive. Only a part of the tongue may be affected either one<br />
or both sides, or the tip or dorsum, thereby giving it a patchy appearance.<br />
Occasionally the affected areas and also the buccal mucosa are covered<br />
with patches of a yellowish grey membranous ulceration. Somewhat<br />
rarely the tongue has a fissured appearance.<br />
Lesions sometimes occur at the angles of the mouth.<br />
The general symptoms vary, typical features being persistent diarrhoea<br />
with frothy light coloured stools and weakness.<br />
Purpura<br />
Haemorrhages from mucous membranes and into the skin and joints<br />
can be classified into symptomatic or non-thrombocytopenic purpura and<br />
thrombocytopenic purpura.<br />
Symptomatic or non-thrombocytopenic purpura.-In one type the<br />
probable pathology is toxic damage to cells of the capillary walls allowing<br />
the extravasation of red blood corpuscles. It is observed in various<br />
infections, toxic disturbances, vitamin C and P deficiency, and in<br />
hereditary familial purpura simplex.<br />
Another type is due to a deficiency of the normal clotting elements of<br />
the blood such as prothrombin, fibrinogen, vitamin K, or to qualitative<br />
changes in the blood platelets.<br />
Thrombocytopenic purpura.-In this condition there is a marked<br />
reduction in the number of platelets in the blood from the normal<br />
400,000 or 500,000 per cmm. to 60,000 per cmm. or even less. The<br />
bleeding time is prolonged to several times the normal level of four<br />
minutes, though the clotting time is unaffected. There is defective clot<br />
retraction and a positive tourniquet test. Acute and chronic recurrent<br />
forms are observed.<br />
The oral manifestations of purpura are of varying severity. The<br />
symptomatic type may only show petechiae and blebs containing blood<br />
in the gingivae or other parts of the mucosa. In the severe and<br />
haemorrhagic types the gingivae are swollen and purplish, and pressure<br />
from dentures may even induce bleeding. There is bleeding from other<br />
mucous membranes, and petechiae and ecchymoses occur on the skin.<br />
The patient has the features arising from loss of blood.<br />
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ORAL <strong>MANIFESTATIONS</strong> <strong>IN</strong> <strong>SYSTEMIC</strong> <strong>DISEASES</strong><br />
Extraction of teeth should be avoided in severe cases whenever possible.<br />
but if essential a time should be chosen when there is remission of<br />
symptoms, and suitable precautions should be taken.<br />
Haemophilia<br />
True haemophilia is a hereditary disease of a sex linked Mendelial<br />
character, that is only transmitted through the female, though the<br />
symptoms only occur in the male. It is considered to be due to a<br />
deficiency of thromboplastinogen in the blood (Quick, 1947). The<br />
coagulation time of the blood is greatly prolonged though the bleeding<br />
time is not unduly affected. There is eventually normal clot retraction.<br />
The tourniquet test is negative.<br />
The gingivae and nasal mucosa are liable to haemorrhage. The<br />
patient is very subject to ecchymoses; also haemorrhages occur into the<br />
joints which become swollen and tender. There may be all the features<br />
of anaemia.<br />
Extraction of teeth must be avoided and only performed as a last<br />
resort. In this case adequate precautions must be undertaken.<br />
Agranulocytosis<br />
The onset of this disease in a number of cases has been associated<br />
with taking drugs of the amidopyrine series. It has also been reported<br />
following the administration of the barbiturates, sulphonamides<br />
(Marshall, 1950), neoarsphenamine (McGibbon and Glyn-Hughes, 1943),<br />
gold preparations, and a case has occurred during penicillin therapy<br />
(Spain and Clarke, 1946).<br />
The blood picture shows a considerable reduction in the granulocytes;<br />
the white count is low and the polymorphonuclears may be 5 per cent.<br />
or under.<br />
The oral lesions are a characteristic feature of the disease. The mucous<br />
membranes of the fauces, the gingivae and buccal mucosa become swollen<br />
and inflamed and may be covered with an exudate. Severe ulceration<br />
may occur involving the fauces, posterior wall of the pharynx, soft and<br />
hard palates, oral mucosa and gingivae. The organisms act rapidly<br />
upon the tissues because of the lack of phagocytic granulocytes and the<br />
lesions progress rapidly to necrosis.<br />
The rectum and vagina may be similarly affected.<br />
The onset is sudden and accompanied by a raised temperature. There<br />
are severe constitutional symptoms and the mortality rate is from 50 per<br />
cent. to 80 per cent.<br />
Leukaemias<br />
Under the term leukaemia is included a variety of conditions in which<br />
the white blood corpuscles are affected, both in number and in form. The<br />
cause is unknown.<br />
241
H. H. STONES<br />
The leukaemias can be classified depending on whether the condition<br />
is acute or chronic and on the type of cell involved; also there is the<br />
aleukaemia phase to be considered. They all have a fatal termination.<br />
(a) Acute leukaemia (myelogenous, lymphatic and monocytic types).<br />
In acute leukaemia the total white count may not be high at the beginning<br />
but is raised to an extent that may vary from 20,000 to upwards of<br />
100,000 per cmm. in the terminal stages.<br />
In the myelogenous type the predominant cells are the primitive<br />
myeloblasts and myelocytes, these forming 80-90 per cent. of the count.<br />
In the lymphatic type, lymphoblasts and lymphocytes similarly form<br />
some 90 per cent. of the count.<br />
In the monocytic type, the monocytes predominate.<br />
There is usually a thrombocytopenia.<br />
The disease most frequently occurs in childhood. The oral mucosa is<br />
affected at an early stage. Frequently the gingivae are swollen and<br />
tend to bleed. They become dark red in colour as the condition<br />
progresses and there may be a tendency to slow but continuous<br />
haemorrhage. Ulcerative lesions develop in the gingivae, palate and<br />
fauces which become secondarily infected and rapidly progress to<br />
gangrene.<br />
Biopsy of the gums shows infiltration with the primitive cells. Burket<br />
(1944) states that the extensive sloughing and occasionally observed<br />
periapical abscess are due to thrombosis of the vessels supplying these<br />
parts.<br />
There are severe haemorrhages from other mucous membranes and<br />
pyrexia and bleeding into the skin. As a result of the continuous<br />
haemorrhages, signs of secondary anaemia develop with a low haemoglobin<br />
and colour index. Eventually there are petechiae and ecchymoses<br />
on the skin.<br />
Neither the spleen nor lymph nodes are markedly enlarged in the acute<br />
conditions. The termination is fatal in either several weeks or months.<br />
Fitzgerald (1943) has reported nine cases and Matheson (1949) four<br />
cases of leukaemia.<br />
Two patients have come under observation who demonstrate the<br />
importance of these diseases to the dental practitioner. The first is of<br />
interest as the gingival condition has been the first symptom of acute<br />
leukaemia. The patient, a female aged 16 years, attended because of<br />
bleeding from the gums on the slightest provocation. There was a<br />
history of haemorrhage following a previous tooth extraction. Examination<br />
revealed swelling of the gingivae. The patient was pale, but stated<br />
she felt well.<br />
Blood examination showed an acute leukaemia with an almost daily<br />
change from a monocytic to myelogenous type. The termination was<br />
fatal in four weeks' time.<br />
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ORAL <strong>MANIFESTATIONS</strong> <strong>IN</strong> <strong>SYSTEMIC</strong> <strong>DISEASES</strong><br />
The second patient was a male aged 16 years who developed acute<br />
myelogenous leukaemia following tooth extraction, though there had<br />
previously been no sign of the disease. There was marked enlargement<br />
and ulceration of the gingivae with gangrene of the soft palate and fauces,<br />
which sloughed away. There was a continuous and intractable oozing of<br />
blood from the oral tissues. The blood picture was typical of<br />
myelogenous leukaemia. The patient only survived for several weeks.<br />
(b) Chronic leukaemia (myelogenous and lymphatic types).-In chronic<br />
myelogenous leukaemia the white cell count shows an increase up to<br />
200,000 or even to 500,000 per cmm. In the early stages adult<br />
polymorphonuclears predominate though myeloblasts and myelocytes are<br />
present.<br />
In chronic lymphatic leukaemia the white cell count may reach 50,000<br />
to 100,000 per cmm. of which some 90 per cent. are small lymphocytes<br />
with occasional lymphoblasts.<br />
In both types the primitive cells gradually increase until the blood,<br />
after a varying period of time, presents the features of the acute type.<br />
Chronic leukaemias frequently occur in middle age.<br />
In both types the onset is gradual. Hypertrophic gingivitis occurs<br />
but the gingival lesions are not so striking unless an acute stage develops<br />
and then there is the tendency to the gingival features already described.<br />
In chronic myelogenous leukaemia the characteristic feature is a<br />
considerably enlarged spleen. In the lymphatic type there is enlargement<br />
of the lymph nodes and the spleen is also sometimes increased in size.<br />
(c) Aleukaemic leukaemia.-In this condition the total number of<br />
white cells is low and sometimes may only reach 1,000 per cmm., but<br />
immature cells are present which may be either of the myoblast,<br />
lymphoblast or monoblast type. These cases develop into one of the<br />
typical leukaemias described above.<br />
A case with unilateral swelling and pain of the jaw and hypertrophic<br />
gingivitis of the same side has been reported by Neger (1939).<br />
Infectious Mononucleosis<br />
In glandular fever there is a leucocytosis of 10,000 to 30,000 cells per<br />
cmm. Lymphocytes, many of which show the morphology of<br />
" monocytoid" cells, eventually form from 60 to 90 per cent. of the<br />
total, though this may not be the case in the early stages.<br />
It is characterized by respiratory symptoms and temperature. There<br />
may be oedema and inflammation of the pharynx, soft palate and uvula,<br />
and ulceration of the fauces. Ravenna and Snyder (1948) have reported<br />
the oral mucosa as being sometimes inflamed though to a lesser extent,<br />
together with the other features in a group of young adults. There is<br />
enlargement of the lymph nodes, especially the cervical group.<br />
243
H. H. STONES<br />
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244