Oral submucous fibrosis is a chronic,complex,irreversible,highly potent pre-cancerous condition characterized by juxta-epithelial inflammatory reaction and progressive fibrosis

Fibrosis

Fibrosis is the formation of excess fibrous connective tissue in an organ or tissue in a reparative or reactive process. This is as opposed to formation of fibrous tissue as a normal constituent of an organ or tissue...

 of the submucosal tissues

Mucous membrane

The mucous membranes are linings of mostly endodermal origin, covered in epithelium, which are involved in absorption and secretion. They line cavities that are exposed to the external environment and internal organs...

 (lamina propria

Lamina propria

The lamina propria is a constituent of the moist linings known as mucous membranes or mucosa, which line various tubes in the body ....

 and deeper connective tissues). As the disease progresses, the jaws become rigid to the point that the sufferer is unable to open his mouth. The condition is linked to oral cancer

Oral cancer

Oral cancer is a subtype of head and neck cancer, is any cancerous tissue growth located in the oral cavity. It may arise as a primary lesion originating in any of the oral tissues, by metastasis from a distant site of origin, or by extension from a neighboring anatomic structure, such as the...

s and is associated with areca nut chewing, the main component of betel quid. Areca nut or betel quid chewing, a habit similar to tobacco chewing

Chewing tobacco

Chewing tobacco Chewing tobacco Chewing tobacco (also known colloquially as hoobastank, backy, tobac, doogooos,Hogleg, chewpoos, chits, chewsky, chawsky, dip, flab, chowers, guy, or a wad, as well as referred to as dipsky, snuff, a pinch, a yopper, a Packing a bomb, a tobbackey or packing a...

, is practiced predominately in Southeast Asia and India, dating back thousands of years

History

In 1952, J. Schwartz coined the term atrophica idiopathica mucosa oris to describe an oral fibrosing disease

Fibrosis

Fibrosis is the formation of excess fibrous connective tissue in an organ or tissue in a reparative or reactive process. This is as opposed to formation of fibrous tissue as a normal constituent of an organ or tissue...

 he discovered in five Indian women from Kenya. S.G. Joshi subsequently coined the termed oral submucous fibrosis (OSF) for the condition in 1953.

Classification

Oral submucous fibrosis is clinically divided into 3 stages (Pindborg J.J.):

Stage 1: Stomatitis

Stage 2: Fibrosis

a- Early lesions, blanching of the oral mucosa

b- Older lesions, vertical and circular palpable fibrous bands in and
around the mouth or lips, resulting in a mottled, marble-like appearance
of the buccal mucosa

Stage 3: Sequelae of oral submucous fibrosis

a- Leukoplakia

b- Speech and hearing deficits
Khanna and Andrade in 1995 developed a group classification system for the surgical management of trismus:

Group I: Earliest stage without mouth opening limitations with an interincisal distance of greater than 35 mm.

Group II: Patients with an interincisal distance of 26-35 mm.

Group III: Moderately advanced cases with an interincisal distance of 15-26 mm. Fibrotic bands are visible at the soft palate, and pterygomandibular raphe and anterior pillars of fauces are present.

Group IVA: Trismus is severe, with an interincisal distance of less than 15 mm and extensive fibrosis of all the oral mucosa.

Group IVB: Disease is most advanced, with premalignant and malignant changes throughout the mucosa.

Pathogenesis

Chronic exposure to beetel nuts, chilli, pepper and prolonged deficiency of iron and zinc may lead to an alteration in oral mucosa, which causes hypersensitivity to these irritants.

This hypersensitivity reaction may often results in a juxta-epithelial inflammation that leads to increased fibroblastic activity resulting in formation of collagen fibres in lamina propria.

These collagen fibers are non degradable and the phagocytic activity is minimized

Symptoms

In the initial phase of the disease, the mucosa feels leathery with palpable fibrotic bands. In the advanced stage the oral mucosa loses its resiliency and becomes blanched and stiff. The disease is believed to begin in the posterior part of the oral cavity and gradually spread outward.

Other features of the disease include:

• Xerostomia
• Recurrent ulceration
• Pain in the ear or deafness
• Nasal intonation of voice
• Restriction of the movement of the soft palate
• A budlike shrunken uvula
  Uvula
  The palatine uvula, usually referred to as simply the uvula , is the conic projection from the posterior edge of the middle of the soft palate, composed of connective tissue containing a number of racemose glands, and some muscular fibers .-Function in language:The uvula plays a role in the...
  
  
• Thinning and stiffening of the lips
• Pigmentation of the oral mucosa
• Dryness of the mouth and burning sensation
• Decreased mouth opening and tongue protrusion

Causes

Dried products such as paan masala

Paan

Paan, from the word pān is an Indian, Pakistani, Uttarvarshi and Southeast Asian tradition of chewing betel leaf with areca nut and slaked lime paste, and katha brown powder paste, with many regional and local variations...

 and gutkha have higher concentrations of areca nut and appear to cause the disease

• Excessive consumption of red chiles
  Chili pepper
  Chili pepper is the fruit of plants from the genus Capsicum, members of the nightshade family, Solanaceae. The term in British English and in Australia, New Zealand, India, Malaysia and other Asian countries is just chilli without pepper.Chili peppers originated in the Americas...
  
  
• Immunological diseases
• Extreme climatic conditions
• Prolonged deficiency to iron and vitamins in the diet

Common sufferers

The incidence of the disease is higher in people from certain parts of the world including South-East Asia, South Africa and Middle East.

Treatment

Biopsy screening is mandatory before treatment. Treatment includes:

• Abstention from chewing areca nut (also known as betel nut) and tobacco
  Tobacco
  Tobacco is an agricultural product processed from the leaves of plants in the genus Nicotiana. It can be consumed, used as a pesticide and, in the form of nicotine tartrate, used in some medicines...
  
  
• Minimizing consumption of spicy foods, including chiles
  Chili pepper
  Chili pepper is the fruit of plants from the genus Capsicum, members of the nightshade family, Solanaceae. The term in British English and in Australia, New Zealand, India, Malaysia and other Asian countries is just chilli without pepper.Chili peppers originated in the Americas...
  
  
• Maintaining proper oral hygiene
  Oral hygiene
  Teeth cleaning is part of oral hygiene and involves the removal of dental plaque from teeth with the intention of preventing cavities , gingivitis, and periodontal disease. People routinely clean their own teeth by brushing and interdental cleaning, and dental hygienists can remove hardened...
  
  
• Supplementing the diet with foods rich in vitamins A
  Vitamin A
  Vitamin A is a vitamin that is needed by the retina of the eye in the form of a specific metabolite, the light-absorbing molecule retinal, that is necessary for both low-light and color vision...
  
  , B complex
  B vitamins
  B vitamins are a group of water-soluble vitamins that play important roles in cell metabolism. The B vitamins were once thought to be a single vitamin, referred to as vitamin B . Later research showed that they are chemically distinct vitamins that often coexist in the same foods...
  
  , and C
  Vitamin C
  Vitamin C or L-ascorbic acid or L-ascorbate is an essential nutrient for humans and certain other animal species. In living organisms ascorbate acts as an antioxidant by protecting the body against oxidative stress...
  
   and iron
  Dietary mineral
  Dietary minerals are the chemical elements required by living organisms, other than the four elements carbon, hydrogen, nitrogen, and oxygen present in common organic molecules. Examples of mineral elements include calcium, magnesium, potassium, sodium, zinc, and iodine...
  
  
• Employing a dental surgeon to round off sharp teeth and extract third molars

Treatment also includes following:

• The prescription of chewable pellets of hydrocortisone
  Cortisol
  Cortisol is a steroid hormone, more specifically a glucocorticoid, produced by the adrenal gland. It is released in response to stress and a low level of blood glucocorticoids. Its primary functions are to increase blood sugar through gluconeogenesis; suppress the immune system; and aid in fat,...
  
   (Efcorlin); one pellet to be chewed every three to four hours for three to four weeks
• Forgoing hot fluids like tea, coffee
• Forgoing alcohol
• Submucosal injections of hydrocortisone 100 mg once or twice daily depending upon the severity of the disease for two to three weeks
• Submucosal injections of human chorionic gonadotrophins
  Human chorionic gonadotropin
  Human chorionic gonadotropin or human chorionic gonadotrophin is a glycoprotein hormone produced during pregnancy that is made by the developing embryo after conception and later by the syncytiotrophoblast .. Some tumors make this hormone; measured elevated levels when the patient is not...
  
   (Placentrax) 2-3 ml per sitting twice or thrice in a week for three to four weeks
• Surgical treatment is recommended in cases of progressive fibrosis when interincisor distance becomes less than 2 centimetre (0.78740157480315 in).(Multiple release incisions deep to mucosa, submucosa and fibrotic tissue and suturing the gap or dehiscence
  Wound dehiscence
  Wound dehiscence is a surgical complication in which a wound breaks open along surgical suture. Risk factors are age, diabetes, obesity, poor knotting or grabbing of stitches, and trauma to the wound after surgery.-Symptoms:...
  
   so created by mucosal graft obtained from tongue and Z-plasty
  Z-plasty
  Z-plasty is a plastic surgery technique that is used to improve the functional and cosmetic appearance of scars. It can elongate a contracted scar or rotate the scar tension line. The middle line of the Z-shaped incision is made along the line of greatest tension or contraction, and triangular...
  
  . In this procedure multiple deep z-shaped incisions are made into fibrotic tissue and then sutured in a straighter fashion)

• Pentoxifylline (Trental), a methylxanthine derivative that has vasodilating properties and increases mucosal vascularity, is also recommended as an adjunct therapy in the routine management of oral submucous fibrosis.

The treatment of patients with oral submucous fibrosis depends on the degree of clinical involvement. If the disease is detected at a very early stage, cessation of the habit is sufficient. Most patients with oral submucous fibrosis present with moderate-to-severe disease. Moderate-to-severe oral submucous fibrosis is irreversible. Medical treatment is symptomatic and predominantly aimed at improving mouth movements.

Stem cell therapy for oral submucosal fibrosis

Recently scientists have proven that intralesional injection of autologous bone marrow stem cells is a safe and effective treatment modality in oral sub mucosal fibrosis. It has been shown autologous bone marrow stem cell injections induces angiogenesis

Angiogenesis

Angiogenesis is the physiological process involving the growth of new blood vessels from pre-existing vessels. Though there has been some debate over terminology, vasculogenesis is the term used for spontaneous blood-vessel formation, and intussusception is the term for the formation of new blood...

in the area of lesion which in turn decreases the extent of fibrosis thereby leading to significant increase in mouth opening.

External links

• http://emedicine.medscape.com/article/1077241-overview
• Stem Cell Therapy for Oral Submucosal Fibrosis in India
• Dentistry Diary
• http://www.maxillofacialcenter.com/BondBook/softtissue/submucousfibrosis.html#Treatment
• http://www.waent.org/archives/2009/vol2-1/submucosal-fibrosis/submucous-fibrosis.htm
• http://www.wrongdiagnosis.com/o/oral_submucous_fibrosis/treatments.htm