Oral submucous fibrosis - Review and case report

M. R. E. Mostehy, BDS, FDSRCS, A.A. Al-Jassem, BDS,MS,

Oral submucous fibrosis is a disease that involves the oral mucosa and produces fibrous changes in several sites of the mouth. It could lead to severe oral deformities with inability to open the mouth, tongue depapillation, and sometimes horseness of voice. Clinical importance of the disease is due to two reasons, namely: i) it is a disease that results from the use of betel-nut chewing in Indians as well as other nationalities adopting the oral habit of betel-nut chewing, ii) it is generally accepted to be a premalignant condition of the oral cavity.This paper reviews the disease and presents a severe case of this malady recorded in an Indian who chews pan-supari.

It is generally recognized that submucous fib­ rosis of the oral cavity is a collagen disease with a great resemblance to morphea or localized scleroderma.1 Although common in Indians, yet it has been reported to occur in other nationalities as sporadic cases.2-3

The disease is characterized by progressive fib­ rosis involving the mucous membrane of the
mouth, mainly the buccal mucosa, soft palate, lip mucosa, and anterior pillar of the fauces. It rarely affects the membrane lining of the pharyngeal box, or vocal cords, but capable of involving the eustac­ hian tube.4

In 1952, Schwartz5 discovered a disease in five Indian patients. He called the disease as "atrophia idiopathica mucosae oris".

The same disease was described by Pindborg & Sirsat1 as an insiduous, chronic disease affecting any part of the mouth and, sometimes, the pharynx. Although occasionally preceded by and associated with vesicle formation, the disease is a juxta-epithelial inflammatory reaction followed
by a fibroblastic change of the lamina propria and epithelial atrophy leading to stiffness of the oral mucosa and, thus, causing trismus and inability to eat.It has been reported that this disease is quite pre­ valent in females6 occuring mostly in the third dec­ ade of life although 2-4-year-old patients have been reported to suffer from it.7 

Etiology

The etiology of oral submucous fibrosis is unknown. However, due to the prevalence of the disease among Indians, a dietary habit3'8 has been incriminated.

The survey performed by Hammer et al4 has shown the cause and effect relationship of submuc­ ous fibrosis and the consumption of spices and pan supari. Although it is not proven that a consumer of these materials necessarily develops the disease, yet, for submucous fibrosis to develop, it appears that an allergic background must be present in the patients.9

The habit of chewing pan supari is prevalent in India, and among Indians who have migrated from other countries and retain the habit. The betel chew, pan supari, comes from a palm called areca catechu. The palm is cultivated throughout tropical India, bears both male and female flowers and yielding a dry fruit which tastes bitter. It is used as a laxative, ft is known to improve appetite and remove foul breath. Traditionally, it is claimed to increase salivation, strengthen the gum, and to pro­ duce mild exhilaration.

The nut of areca catechu contains several alkaloids, such as arecaidine, arecoline, arecaine, guvacine, and guvacoline.9

The user takes fragments from the core of pan supari and adds crushed pieces of marble, or
slaked lime (calcium hydroxide), then wraps all components in a leaf of piper betel vine after soaking the contents in lemon juice (Fig. 1). Some prefer to leave the wrapped leaf to dry, while others insert it directly into the sulcus of the lower jaw. Sometimes, other additives (tobacco, cardamom, cloves and camphor) are used to give the mixture a better aroma. The leaves of piper betel contain an alkaloid "arakene" with properties similar to cocaine.10

The components of the leaf are considered as a strong astringent besides other functions as being digestive, antiseptic, carminative, and aphrodisiac. It is also used internally as an antidote to snakebites.

When the mixture is chewed, it gives the person a disagreeable burning taste and a feeling of throat constriction. After the first effect of the salivary glands' excitation and severe irritation of the oral mucosa, a pleasant odor remains in the mouth. Persons using this chew for the first time, experi­ ence some cerebral effects like uneasiness, faint-ness, slight excitation, and sweating.

Some recent investigations,11'2 have proven that there is a high cause and effect relationship between using pan supari and submucous fibrosis. Although the tissue changes might result from chronic and prolonged irritation by these agents is considered an allergic effect or an altered dietary habit, as an aggravating factor in the development of oral submucous fibrosis.

Cannif and Harvey12 performed in vitro studies on the effect of the alkaloid component of areca catechu on the growth rate of human fibroblasts. They found that collagen formation was 170% higher than the control cultures. They deduced that the continuous use of pan supari, as a habit by Indians, might produce increased collagen forma­ tion that undergoes hyalinization and fibrosis.

Signs and Symptoms

In the early stages, and in youngsters in particular, the early symptoms are burning sensation, with dry­ ness of the oral cavity. Vesicular eruptions occur at any time and may be located on any mucosal sur­ face, especially the buccal mucosa, soft palate, or the labial mucosa. Once they erupt, minute mucosal erosions occur which are very painful.

As the condition advances, the oral mucosa shows a patchment-like leathery appearance that eventually changes into a marble-like texture. This leads to bands of fibrous tissue that give a firm tex­ ture on palpation and inability to open the mouth to its maximum extent.13

The labial mucosa becomes whitish, ischemic, tense, and non-pliable. Early, there are reddish areas alternating with whitish bands, but later the labial mucosa gains totally the marble-like appear­ ance. The vermilion border of the lips becomes atrophied with possible patchy hyper-pigmented areas.In the progress of the disease, the tongue becomes fixed and there is a total loss of the papil­ lae, with whitening and board-like bald appear­ance. Opening the mouth, thus, gets markedly reduced; and this is in direct relationship with the amount of submucous fibrosis.

Case Report

A 39-year-old Indian male was referred to the Department of Periodontics, Dental Center in Kuwait complaining of inability to open his mouth. He indicated that he was an early user of pan supari since he was 12 years old, and that he would have continued chewing same if it were available in Kuwait. Other complaints were that he was not able to use the toothbrush; and hot and spicy food caused him a great deal of discomfort.

On examination, the mouth maximally opened with an inter-incisal distance of 2.3 cms (Fig. 2).
The oral mucosa was whitish, with areas of deline­ ation on both iips and their vestibular mucosa (Figs. 3,4).

Tongue mobility was greatly reduced and the mucosa! covering showed patchy scarred areas,
with partial depapillation along the entire dorsal surface (Fig. 5). The cheek mucosa was also mar-ble-like and tense. Fibrous bands could be felt along the entire area of the cheek mucosa.

On palpation, there was a tense feeling of the oral aperture with marked atrophy of the labial and cheek tissue bulk. Blood biochemistry profiles were within the normal values. Hemoglobin con­ centration was low at 7.8 gm/100 ml.

Lip biopsy from the involved area showed severe epithelial atrophy with mild hyperkeratosis. The lamina propria showed marked collagen bundle condensation. In some parts, marked hyalinization of the sub-epithelial connective tissue was noticed (Fig. 6).

It is generally agreed that submucous fibrosis, once established, becomes irreversible14 and pro­ duces progressive stiffening of the oral mucosa. Cases involving other areas like the vocal cords, eustachian tube, anterior pillars of the fauces, and mucous membrane of the pharynx have been reported.6 Such cases produce other manifesta­tions as hoarse-ness of voice, difficulty in swallow­ ing, as well as impaired hearing. Some cases, when affected by unilateral submucous fibrosis, will lead to deviation of the mandible on opening towards the affected side.8

Vesicles may precede or follow the changes in the underlying connective tissue. The sub-epithe-lial layer may become hyalinized in addition to the severe epithelial atrophy that involves up to 90% of the cases.3

Submucous fibrosis was considered as a pre-malignant condition by several authors6-14-16 due to the fact that oral leukoplakia occurred in a high incidence among patients with submucous fibrosis.6 In Pindborg's report17 of 268 cases of sub­ mucous fibrosis, in India, leukoplakia occurred in 55%. Also, in 200 cases of frank oral cancer, 89% had submucous fibrosis prior to any visible car­ cinomatous changes. It is speculated that submucous fibrosis produces fibroblastic degeneration in the underlying connective tissue and, hence, an eventual degeneration of the epithelial cells which, in turn, provides a predisposing situation for the action of carcinogen S, the most important being tobacco.11

With respect to the treatment of submucous fib­ rosis, several conservative approaches have been tried with little success.4'6'8 All therapies strictly advise abstinence from the bad habit Palliative treatment, e.g., stretching exercise, balancing the patient's diet, and regular check-ups of the patient's oral condition is directed towards alleviat­ing the resulting complications.In the present case, the patient was asked to ref­ rain from the habitand report for check-up atthree-month intervals.

Address reprint requests to:

Dr.M.R.EI-Mostehy,

Kuwait Dental Center,

 Sharq 13041, Kuwait.