Orofacial granulomatosis

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Orofacial granulomatosis
Other namesgranulomatous cheilitis, cheilitis granulomatosa, cheilitis granulomatosis, oral granulomatosis
Specialty Gastroenterology   OOjs UI icon edit-ltr-progressive.svg

Orofacial granulomatosis (OFG) is a condition characterized by persistent enlargement of the soft tissues of the mouth, lips and the area around the mouth on the face, causing in most cases extreme pain. The mechanism of the enlargement is granulomatous inflammation. [1] The underlying cause of the condition is not completely understood, and there is disagreement as to how it relates to Crohn's disease and sarcoidosis. [1]

Contents

Signs and symptoms

Signs and symptoms may include:

The enlargement of the tissues of the mouth, lips and face seen in OFG is painless. [5] Melkersson–Rosenthal syndrome is where OFG occurs with fissured tongue and paralysis of the facial nerve. [5] The cause of the facial paralysis is thought to be caused by the formation of granulomas in the facial nerve, which supplies the muscles of facial expression. [2]

Causes

The cause of the condition is unknown. [6] [3] The disease is characterized by non-caseating granulomatous inflammation. [1] That is, the granulomas do not undergo the caseating ("cheese-like") necrosis typical of the granulomas of tuberculosis.

There is disagreement as to whether OFG represents an early form of Crohn disease or sarcoidosis, or whether it is a distinct, but similar clinical entity. [1] [7] Crohn disease can affect any part of gastrointestinal tract, from mouth to anus. When it involves the mouth alone, some authors refer to this as "oral Crohn disease", distinguishing it from OFG, and others suggest that OFG is the same condition as Crohn disease when it presents in the oral cavity.

OFG may represent a delayed hypersensitivity reaction, but the causative antigen(s) is not identified or varies form one individual to the next. Suspected sources of antigens include metals, e.g. cobalt, or additives and preservatives in foods, including benzoates,benzoic acid,cinnamaldehyde,metabisulfates,butylated hydroxyanisole,dodecyl gallate,tartrazine, or menthol, [3] [6] [8] Examples of foods which may contain these substances include margarine, cinnamon, eggs, chocolate or peppermint oil. [3] [6] [9]

Some suggest that infection with atypical mycobacteria could be involved, (paratuberculosis), [3] and that OFG is a reaction to mycobacterial stress protein mSP65 acting as an antigen. [6]

In response to an antigen, a chronic, submucosal, T cell mediated inflammatory response occurs, which involves cytokines (e.g. tumor necrosis factor alpha), protease-activated receptors, matrix metalloproteinases and cyclooxygenases. [6] The granulomas in OFG form in the lamina propria, and may form adjacent to or within lymphatic vessels. [8] This is thought to cause obstruction of lymphatic drainage and lymphedema which is manifest as swelling clinically. [6]

There may be a genetic predisposition to the condition. [6] People who develop OFG often have a history of atopy, such as childhood asthma or eczema. [3]

Diagnosis

The diagnosis is usually made by tissue biopsy, however this cannot reliably distinguish between the granulomas of OFG and those of Crohn disease or sarcoidosis. [8] Other causes of granulomatous inflammation are ruled out, such as sarcoidosis, Crohn disease, allergic or foreign body reactions and mycobacterial infections. [5]

Classification

OFG could be classified as a type of cheilitis (lip inflammation), hence the alternative names for the condition using the word cheilitis, and a granulomatous condition.

Treatment

Anti-tumour necrosis factor α drugs (e.g. infliximab) [10]

Dietary restriction of a particular suspected or proven antigen may be involved in the management of OFG, such as cinnamon or benzoate-free diets. [8]

Epidemiology

OFG is uncommon, but the incidence is increasing. [2] The disease usually presents in adolescence or young adulthood. [6] It may occur in either sex, but males are slightly more commonly affected. [6]

History

OFG was first described in 1985. [1]

Related Research Articles

Mouth ulcer Medical condition

A mouth ulcer (aphtha) is an ulcer that occurs on the mucous membrane of the oral cavity. Mouth ulcers are very common, occurring in association with many diseases and by many different mechanisms, but usually there is no serious underlying cause. Rarely, a mouth ulcer that does not heal may be a sign of oral cancer. These ulcers may form individually or multiple ulcers may appear at once. Once formed, an ulcer may be maintained by inflammation and/or secondary infection.

Granuloma Medical condition

A granuloma is an aggregation of macrophages that forms in response to chronic inflammation. This occurs when the immune system attempts to isolate foreign substances that it is otherwise unable to eliminate. Such substances include infectious organisms including bacteria and fungi, as well as other materials such as foreign objects, keratin, and suture fragments.

Granulomatosis with polyangiitis Medical condition

Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), is a rare long-term systemic disorder that involves the formation of granulomas and inflammation of blood vessels (vasculitis). It is a form of vasculitis that affects small- and medium-size vessels in many organs but most commonly affects the upper respiratory tract, lungs and kidneys. The signs and symptoms of GPA are highly varied and reflect which organs are supplied by the affected blood vessels. Typical signs and symptoms include nosebleeds, stuffy nose and crustiness of nasal secretions, and inflammation of the uveal layer of the eye. Damage to the heart, lungs and kidneys can be fatal.

Uveitis Inflammation of the uvea of the eye

Uveitis is the inflammation of the uvea, the pigmented layer that lies between the inner retina and the outer fibrous layer composed of the sclera and cornea. The uvea consists of the middle layer of pigmented vascular structures of the eye and includes the iris, ciliary body, and choroid. Uveitis is described anatomically, by the part of the eye affected, as anterior, intermediate or posterior, from front to back. In the panuveitic form, all parts are involved. The commonest is the anterior form. Symptoms include pain, floaters and blurred vision, and clinical examination may show redness and an irregular pupil, while opthalmic examination shows dilated ciliary blood vessels and the presence of cells in the anterior chamber. Uveitis may arise spontaneously and associated with some genetic factors, or be associated with a wide range of conditions including autoimmune disease and infections. While the eye is a relatively protected environment, its immune mechanisms may be overcome resulting in inflammation and tissue destruction associated with T-cell activation. The incidence is approximately 1:4500, most commonly between the ages of 20–60.

Gastrointestinal disease Medical condition

Gastrointestinal diseases refer to diseases involving the gastrointestinal tract, namely the oesophagus, stomach, small intestine, large intestine and rectum, and the accessory organs of digestion, the liver, gallbladder, and pancreas.

Tuberculous lymphadenitis Medical condition

Tuberculous lymphadenitis is the most common form of tuberculosis infections that appears outside the lungs. Tuberculous lymphadenitis is a chronic, specific granulomatous inflammation of the lymph node with caseation necrosis, caused by infection with Mycobacterium tuberculosis or related bacteria.

Stomatitis Medical condition

Stomatitis is inflammation of the mouth and lips. It refers to any inflammatory process affecting the mucous membranes of the mouth and lips, with or without oral ulceration.

Angular cheilitis Cheilitis characterized by inflammation of one or both of the corners of the mouth

Angular cheilitis (AC) is inflammation of one or both corners of the mouth. Often the corners are red with skin breakdown and crusting. It can also be itchy or painful. The condition can last for days to years. Angular cheilitis is a type of cheilitis.

Melkersson–Rosenthal syndrome Medical condition

Melkersson–Rosenthal syndrome is a rare neurological disorder characterized by recurring facial paralysis, swelling of the face and lips and the development of folds and furrows in the tongue. Onset is in childhood or early adolescence. After recurrent attacks, swelling may persist and increase, eventually becoming permanent. The lip may become hard, cracked, and fissured with a reddish-brown discoloration. The cause of Melkersson–Rosenthal syndrome is unknown, but there may be a genetic predisposition. It has been noted to be especially prevalent among certain ethnic groups in Bolivia. It can be symptomatic of Crohn's disease or sarcoidosis. Approximately 400 cases have been reported worldwide.

An oral medicine or stomatology doctor has received additional specialized training and experience in the diagnosis and management of oral mucosal abnormalities including oral cancer, salivary gland disorders, temporomandibular disorders and facial pain, taste and smell disorders; and recognition of the oral manifestations of systemic and infectious diseases. It lies at the interface between medicine and dentistry. An oral medicine doctor is trained to diagnose and manage patients with disorders of the orofacial region, essentially as a "physician of the mouth."

Sialadenitis Medical condition

Sialadenitis (sialoadenitis) is inflammation of salivary glands, usually the major ones, the most common being the parotid gland, followed by submandibular and sublingual glands. It should not be confused with sialadenosis (sialosis) which is a non-inflammatory enlargement of the major salivary glands.

Cheilitis Inflammation of the lips

Cheilitis is a medical condition characterized by inflammation of the lips. According to its onset and course, cheilitis can be either acute or chronic. Most cheilitis is caused by acute sun exposure. Allergic tests may identify allergens that cause cheilitis.

Gingival enlargement Human disease

Gingival enlargement is an increase in the size of the gingiva (gums). It is a common feature of gingival disease. Gingival enlargement can be caused by a number of factors, including inflammatory conditions and the side effects of certain medications. The treatment is based on the cause. A closely related term is epulis, denoting a localized tumor on the gingiva.

Oral and maxillofacial pathology refers to the diseases of the mouth, jaws and related structures such as salivary glands, temporomandibular joints, facial muscles and perioral skin. The mouth is an important organ with many different functions. It is also prone to a variety of medical and dental disorders.

Persistent edema of rosacea is a hard, nonpitting edema found on the areas involved, those mainly being the forehead, glabella, upper eyelids, nose, and/or cheeks.

Blau syndrome Medical condition

Blau Syndrome is an autosomal dominant genetic inflammatory disorder which affects the skin, eyes, and joints. It is caused by a mutation in the NOD2 (CARD15) gene. Symptoms usually begin before the age of 4, and the disease manifests as early onset cutaneous sarcoidosis, granulomatous arthritis, and uveitis.

Plasma cell gingivitis Medical condition

Plasma cell gingivitis is a rare condition, appearing as generalized erythema (redness) and edema (swelling) of the attached gingiva, occasionally accompanied by cheilitis or glossitis. It is called plasma cell gingivitis where the gingiva (gums) are involved, plasma cell cheilitis, where the lips are involved, and other terms such as plasma cell orifacial mucositis, or plasma cell gingivostomatitis where several sites in the mouth are involved. On the lips, the condition appears as sharply outlined, infiltrated, dark red plaque with a lacquer-like glazing of the surface of the involved oral area.

Cervical lymphadenopathy

Cervical lymphadenopathy refers to lymphadenopathy of the cervical lymph nodes. The term lymphadenopathy strictly speaking refers to disease of the lymph nodes, though it is often used to describe the enlargement of the lymph nodes. Similarly, the term lymphadenitis refers to inflammation of a lymph node, but often it is used as a synonym of lymphadenopathy.

Fungal sinusitis Inflammation of the paranasal sinuses due to fungal infection

Fungal sinusitis is the inflammation of the lining mucosa of the paranasal sinuses due to fungal infection. It occurs in people with reduced immunity. The maxillary sinus is the most commonly involved. Fungi responsible for fungal sinusitis are Aspergillus fumigatus (90%), Aspergillus flavus, and Aspergillus niger. Fungal sinusitis occurs most commonly in middle-aged populations. Diabetes mellitus is the most common risk factor involved.

Oral manifestations of systematic disease are signs and symptoms of disease occurring elsewhere in the body detected in the oral cavity and oral secretions. High blood sugar can be detected by sampling saliva. Saliva sampling may be a non-invasive way to detect changes in the gut microbiome and changes in systemic disease. Another example is tertiary syphilis, where changes to teeth can occur. Syphilis infection can be associated with longitudinal furrows of the tongue.

References

  1. 1 2 3 4 5 Grave, B; McCullough, M; Wiesenfeld, D (January 2009). "Orofacial granulomatosis--a 20-year review". Oral Diseases. 15 (1): 46–51. doi:10.1111/j.1601-0825.2008.01500.x. PMID   19076470.
  2. 1 2 3 4 5 6 7 8 9 10 Leão, JC; Hodgson, T; Scully, C; Porter, S (Nov 15, 2004). "Review article: orofacial granulomatosis". Alimentary Pharmacology & Therapeutics. 20 (10): 1019–27. doi: 10.1111/j.1365-2036.2004.02205.x . PMID   15569103. S2CID   33359041.
  3. 1 2 3 4 5 6 Jordan, Michael A.O. Lewis, Richard C.K. (2012). Oral medicine (Second ed.). London: Manson Publishing. pp. 128–29. ISBN   978-1840761818.
  4. ↑ Welbury R; Duggal M; Hosey MT (2012). Paediatric dentistry (4th ed.). Oxford: Oxford University Press. p. 319. ISBN   978-0199574919.
  5. 1 2 3 Treister NS, Bruch JM (2010). Clinical oral medicine and pathology . New York: Humana Press. pp.  51, 147. ISBN   978-1-60327-519-4.
  6. 1 2 3 4 5 6 7 8 9 Scully C (2013). Oral and maxillofacial medicine : the basis of diagnosis and treatment (3rd ed.). Edinburgh: Churchill Livingstone. pp. 298–301. ISBN   9780702049484.
  7. ↑ Zbar, AP; Ben-Horin, S; Beer-Gabel, M; Eliakim, R (March 2012). "Oral Crohn's disease: is it a separable disease from orofacial granulomatosis? A review". Journal of Crohn's & Colitis. 6 (2): 135–42. doi: 10.1016/j.crohns.2011.07.001 . PMID   22325167.
  8. 1 2 3 4 Scully, Crispian; Oslei Paes de Almeida; Jose Bagan; Pedro Diz Dios; Adalberto Mosqueda Taylor (2010). Oral medicine and pathology at a glance. Chichester, UK: Wiley-Blackwell. ISBN   978-1405199858.
  9. ↑ Woo, Sook-Bin (2012). Oral pathology : a comprehensive atlas and text (1st ed.). Philadelphia, PA: Elsevier/Saunders. pp. 154, 155. ISBN   978-1437722260.
  10. ↑ O'Neill, ID; Scully, C (October 2012). "Biologics in oral medicine: oral Crohn's disease and orofacial granulomatosis". Oral Diseases. 18 (7): 633–38. doi:10.1111/j.1601-0825.2012.01918.x. PMID   22420719.
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