**4.3 Salivary gland**

*Sarcoidosis and Granulomatosis - Diagnosis and Management*

• OFG alone

**3. Etiology**

**4. Presentation**

**4.1 Lips**

rubbery [24].

**4.2 Oral lesions**

clinical features of OFG [22].

• OFG with intestinal CD

tissues in the oral and maxillofacial region that present clinically with labial enlargement, perioral and/or mucosal swelling, oral ulcerations, gingivitis, and a variety of other orofacial features [9]. The clinical manifestations can be highly variable, and this variability makes it difficult to diagnose. OFG is a disease that encompasses a broad range of presentations, which may include oral manifestations of a systemic condition such as Crohn's disease (CD), sarcoidosis, granulomatosis with polyangiitis, and Melkersson-Rosenthal syndrome [10]. On the other hand, some studies say that OFG displays a spectrum of diseases ranging from granulomatous cheilitis to patients with granulomas involving other orofacial tissues, with or without facial nerve palsy and plicated tongue (Melkersson-Rosenthal syndrome) [11]. According to recent evidence, OFG can also be classified into three categories [12, 13], namely:

• OFG with gastrointestinal granulomata but no symptoms of intestinal CD.

The exact cause of OFG is yet to be elucidated, although various etiological agents have been proposed such as genetic predisposition, contact allergies, various microbiological agents, and immunologic causes [9]. The role of genetic predisposition has been evaluated in different studies, but there is a lack of conclusive evidence between HLA and pathogenesis of orofacial granulomatosis [14–16]; there is no evidence to support genetics causes for OFG [16]. Because of involvement of OFG in CD and sarcoidosis, the possible role of infections in the pathogenesis of OFG has been suggested [17]. Several studies have suggested that there is no conclusive evidence to support the role for allergy in OFG [18]. Recently, a monoclonal lymphocytic expansion in OFG lesion has been identified that may be responsible for the granuloma formation through cytokine production in lesions [19, 20].

The diagnosis of OFG is based on clinical presentation, but it can be highly variable. The clinical features of OFG are mainly similar to orofacial manifestations of CD without apparent lesions in the bowels and may also mimic orofacial manifestations of sarcoidosis [21] labial enlargement, and sometimes oral ulcers are the main

The lips are the most common sites of involvement in OFG. Labial swelling can involve the lower or upper lip or both. This feature of OFG is persistent but may eventually become recurrent. Each episode in this inflammatory process usually lasts several weeks to months [23]. The swelling varies in consistency from soft to

Three types of oral ulcers may occur in OFG as follows [25]:

**2**

In asymptomatic patients, enlargement of the salivary glands is the first identifiable sign of the disease [8]. Involvement of salivary glands has been reported in the maxillofacial region that xerostomia or bilateral parotid swelling is the result of this involvement [26, 27]. Inflammation of salivary glands differentiates OFG from other granulomatous diseases such as cheilitis glandularis, Wegener's granulomatosis, sarcoidosis, and deep fungal infections [28].
