Melkersson-Rosenthal syndrome - Oral Mucosal Lesion
The syndrome may be idiopathic, but evaluation for systemic disease is warranted as orofacial granulomatosis may be a presenting sign for underlying conditions such as Crohn disease or sarcoidosis. Granulomatous cheilitis may also be associated with hypersensitivity to some foods and food additives (in particular benzoates and cinnamon products), or sensitivities to amalgam. If no specific etiology can be identified (eg, after patch testing or evaluation for systemic disease), the condition is deemed idiopathic. However, orofacial granulomatosis may be the first manifestation of Crohn disease, with gastrointestinal signs and symptoms occurring years later.
Orofacial granulomatosis typically presents in the late second decade but has been reported in children as young as 5 years. No predilection has been shown for sex or race and ethnicity.
G51.2 – Melkersson's syndrome
37770007 – Melkersson-Rosenthal syndrome
Differential Diagnosis & Pitfalls
- Orofacial granulomatosis – Facial, lip, and oral mucosa swelling and granuloma formation; a broader term that encompasses incomplete forms of Melkersson-Rosenthal syndrome.
- Allergic contact dermatitis (adult, pediatric) / stomatitis – This is almost always associated with redness of the skin or mucosa, or with fissuring, ulceration, or scaling, which is absent in granulomatous cheilitis.
- Angioedema – Acute swelling that completely resolves within 24-48 hours. May have associated airway involvement.
- Ascher syndrome – Inherited condition with swelling of lips and eyelids.
- Crohn disease – Orofacial granulomatosis may precede overt manifestations of Crohn disease by years.
- Infection – This would be acute in onset and acutely painful (see, eg, cellulitis).
- Sarcoidosis – This may present with granulomas of the salivary glands in the lips and gingival swelling.