Presentation
In the present case the lesional tissue was negative for AFB and normal chest radiograph was present. [ijpmonline.org]
[1] The classic presentation of OFG is a nontender recurrent labial swelling that eventually becomes persistent.[2] However, the clinical presentation can be highly variable, making the diagnosis difficult to establish.[3] We report the case of a 61- [ncbi.nlm.nih.gov]
Orofacial granulomatosis is a disease with wide spectrum of clinical presentation. The causative agent in the present case is unknown; hence it is categorized as idiopathic Orofacial granulomatosis. [hindawi.com]
Oral manifestations have a prevalence ranging from 20 to 50%, and initial presenting signs occur in 5-10% of affected patients (fig. 1a). [karger.com]
Entire Body System
- Pain
Treatment is offered where there is pain, cosmetic concerns or impaired function. [dermnetnz.org]
Pain. Trouble using your face or mouth properly. The most common treatment involves corticosteroids. [my.clevelandclinic.org]
Case presentation A 31-year-old white man presented to our Gastroenterology clinic with complaints of painful swelling of the mouth with oral ulcers and difficulty eating. [jmedicalcasereports.biomedcentral.com]
These lesions could cause pain and are a discomfort to the patients; they are not associated with intestinal activity. Nonspecific lesions are not pathognomonic of CD (table 1). [karger.com]
- Lymphadenopathy
] The patient represented a case of idiopathic OFG with histopathologically proven noncaseating granulomas involving the lower lip mucosa, tongue, and the oral cavity with cervical lymphadenopathy. [ncbi.nlm.nih.gov]
The patient was negative for fever, lymphadenopathy, genital ulcers, arthralgia, ocular symptoms and diarrhoea. His familial history was unremarkable, especially with regard to angioedema or chronic inflammatory bowel diseases. [medicaljournals.se]
Pulmonary infiltration and hilar lymphadenopathy are the common presentations. [ijpmonline.org]
The facial skin may be dry, exfoliative (flaking) or erythematous.[2] Cervical lymphadenopathy (enlarged lymph nodes in the neck).[2] Facial palsy (weakness and altered sensation of the face).[2] The enlargement of the tissues of the mouth, lips and face [en.wikipedia.org]
There was no lymphadenopathy palpated. The rest of his examination was unremarkable. Laboratory work revealed a normal hemoglobin, platelet count, and CRP. Mandibular X-rays and an ultrasound showed no fractures or abscesses in the affected area. [jmedicalcasereports.biomedcentral.com]
- Lymphedema
[…] 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 [en.wikipedia.org]
Gastrointestinal
- Abdominal Pain
CD and UC are commonly associated with abdominal pain, diarrhea, rectal bleeding, weight loss and signs of malnutrition [12]. Approximately one third of IBD patients develop extraintestinal manifestations in the course of their disease [13]. [karger.com]
Most common symptoms include long-standing diarrhea, weight loss, and abdominal pain. [ijpmonline.org]
- Diarrhea
CD and UC are commonly associated with abdominal pain, diarrhea, rectal bleeding, weight loss and signs of malnutrition [12]. Approximately one third of IBD patients develop extraintestinal manifestations in the course of their disease [13]. [karger.com]
Most common symptoms include long-standing diarrhea, weight loss, and abdominal pain. [ijpmonline.org]
Jaw & Teeth
- Lip Swelling
Lip swelling is common Lip swelling due to OFG may initially only last a few hours at a time, and can be difficult to distinguish from another type of lip swelling known as angioedema. [allergy.org.au]
One month later, the patient notified us of mild lower lip swelling, but did not visit the hospital. [journals.lww.com]
A: Mild lip swelling, with dry lips. [scielo.br]
It is characterized by subepithelial noncaseating granulomas and has a spectrum of possible clinical manifestations ranging from subtle oral mucosal swelling to permanent disfiguring fibrous swelling of the lips and face. [pubmed.ncbi.nlm.nih.gov]
The most common clinical presentation is painless swelling of one or both lips. Involvement of the lower lip is the most common. Initially, the swelling is soft and comes and goes, with each episode lasting weeks or months. [dermnetnz.org]
- Fissured Tongue
Some people have a fissured tongue, and may sometimes develop facial paralysis. Mouth ulcers and inflammation of the gums, known as gingivitis may be seen. Other symptoms include tongue swelling or a sensation of a burning tongue. [allergy.org.au]
Gingival enlargement (swelling of the gums).[2] Fissured tongue (grooves in the tongue).[2] Enlargement of the mucous membrane of the mouth, which may be associated with cobblestoning and mucosal tags (similar lesions often occur on the intestinal mucosa [en.wikipedia.org]
tongue) Facial nerve paralysis may also be associated with orofacial granulomatosis as in Melkersson-Rosenthal syndrome. [dermnetnz.org]
The fissured tongue is congenital and is usually asymptomatic and benign and does not require any treatment [45]. [karger.com]
Intraoral manifestations include cobblestone appearance of buccal mucosa, ulcers, granulomatous gingivitis, mucosal tags, and fissured tongue rarely. [ijpmonline.org]
- Aphthous Ulceration
Other signs in the mouth of orofacial granulomatosis may include: Painful mouth ulcers – may resemble aphthous ulcers or form chronic linear deep ulcers in the folds between the gums and inside of the cheeks or lips Mucosal tags – usually occur deep in [dermnetnz.org]
Case Report A 14-year-old Caucasian male presented with a 5-month history of permanent painless swelling of the lower lip and, to a lesser extent, the upper lip (Fig. 1a), accompanied by considerable gingival hyperplasia and superficial aphthous ulcers [medicaljournals.se]
- Cracked Lips
Related symptoms may include: Cracked lips. Drooling. Dry, red, peeling skin around your mouth. Grooves or ridges on your tongue. Mouth ulcers or mucosal tags (bumps or small flaps of extra skin on the tissue inside of your mouth). [my.clevelandclinic.org]
Skin
- Erythema
There were two small superficial ulcerations seen in his mid-gastric body and erythema and erosions in the antrum and duodenum. Biopsies revealed nonspecific chronic active gastritis and duodenitis. [jmedicalcasereports.biomedcentral.com]
Clinical Images of a 50-Year-Old Woman With Severe Orofacial Granulomatosis Before and After Azithromycin Pulse Therapy A, Severe erythema, edema, and swelling of the upper lip and both cheeks prior to azithromycin pulse treatment. [jamanetwork.com]
For example, intraoral involvement may take the form of hypertrophy, erythema, or nonspecific erosions involving the gingiva, oral mucosa, or tongue.[6,8] Upon microscopic identification of granulomatous inflammation, special stains are used to rule out [ncbi.nlm.nih.gov]
The most common oral manifestations of GPA are strawberry-like gingivitis, erythema, osteonecrosis of the palate, desquamation and nodular masses of the lips, petechiae and ulceration. [karger.com]
The clinical features of this disease extraorally in the frequency of occurrence are swelling of lips and face which become persistent as the disease progresses, fissuring of lips, angular cheilitis, and perioral erythema. [ijpmonline.org]
- Dermatitis
A lip-licking habit may result in irritant contact dermatitis. The skin around the mouth may become dry, red and peeling. The lip swelling may become severe enough to interfere with speech and eating. [dermnetnz.org]
Am J Contact Dermat. 1997;8:35–8. [PubMed] [Google Scholar] 15. Sciubba JJ, Said-Al-Naief N. Orofacial granulomatosis: Presentation, pathology and management of 13 cases. J Oral Pathol Med. 2003;32:576–85. [PubMed] [Google Scholar] 16. [ncbi.nlm.nih.gov]
Treatment
Epidemiological characteristics, disease profiles, prior treatment histories, and responses to azithromycin treatment of all these patients are summarized in the eTable in the Supplement. [jamanetwork.com]
Topical tacrolimus alone or in combination with other treatments has also been used for mild swelling. Another immunomodulating treatment is often required in addition to the steroid. [dermnetnz.org]
Talk to your healthcare provider if you have any facial swelling so you can be tested and receive treatment. There’s no cure for orofacial granulomatosis, but corticosteroids and other treatments may reduce symptoms. [my.clevelandclinic.org]
Treatment of orofacial granulomatosis: a case report. [jmedicalcasereports.biomedcentral.com]
Prognosis
Outlook / Prognosis What’s the outlook for people with orofacial granulomatosis? Treatment for orofacial granulomatosis is complex. Current strategies may work slowly, over months or years. Sometimes, they don’t help at all. [my.clevelandclinic.org]
Prognosis is good, but patients must be informed that recurrence is common and often unexplained.77 Campbell HE, Escudier MP, Patel P, Challacombe SJ, Sanderson JD, Lomer MC. [scielo.br]
Etiology
Hence, more studies are needed to clarify the role of this spirochete as an etiological factor of OFG. [karger.com]
Spirochetes—the possible etiological factor of the cheilitis granulomatosa. Chin Med Sci J 2001;16:52–5. Cited Here | PubMed [10]. Gibson J, Wray D, Bagg J. [journals.lww.com]
The etiological agents such as food substances, food additives, dental material microbiological agents have been proposed, but its pathogenesis is uncertain. [hindawi.com]
[…] been implicated in inducing tissue changes consistent with OFG.[14] The cause can be a protracted one, ranging over many years.[9] The treatment of OFG is difficult, particularly in the absence of an etiologic factor. [ncbi.nlm.nih.gov]
Epidemiology
Treatment[edit] 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[edit] OFG is [en.wikipedia.org]
Epidemiological characteristics, disease profiles, prior treatment histories, and responses to azithromycin treatment of all these patients are summarized in the eTable in the Supplement. [jamanetwork.com]
Prevention
Prevention How can I prevent orofacial granulomatosis? Because scientists don’t yet understand what causes orofacial granulomatosis, there’s no proven way to prevent it. [my.clevelandclinic.org]
Early diagnosis of OFG is a crucial step to prevent and cure its unsightly sequelae and sometimes avoid progression of systemic potentially life-threatening OFG-related diseases. [onlinelibrary.wiley.com]
Hence followup for the patient is advised as a step in prevention of a possible systemic granulomatous disease. Conflict of Interests The authors declare that they have no conflict of interests. References L. R. [hindawi.com]
Clinical outcome is unpredictable, however, persistent orofacial swelling can be prevented when OFG is diagnosed and treated early on.1717 Thomas TK, Neelakandan RS, Bhargava D, Deshpande A. Orofacial granulomatosis: A clinicopathologic correlation. [scielo.br]