Melkersson-Rosenthal syndrome

name: Melkersson-Rosenthal syndrome
creation_date: '2026-01-20T22:43:10Z'
updated_date: '2026-02-17T21:53:14Z'
category: Genetic
disease_term:
  preferred_term: Melkersson-Rosenthal syndrome
  term:
    id: MONDO:0007969
    label: Melkersson-Rosenthal syndrome
parents:
- Granulomatous disease
- Autoinflammatory syndrome
epidemiology:
- name: Age of Onset
  description: Typical age range for Melkersson-Rosenthal syndrome presentation
  minimum_value: 14
  maximum_value: 39
  mean_range: Second decade of life (10-20 years)
  unit: years
  notes: >
    Mean onset frequently in the second decade. However, cases have been reported
    across a wide age range from childhood to adulthood.
    The classic triad may not be fully apparent at disease onset, leading to delayed
    diagnosis.
  evidence:
  - reference: PMID:31466978
    supports: SUPPORT
    snippet: "he exhibited recurrent facial nerve palsy since the age of 10 years"
    explanation: "Documents MRS onset in childhood/adolescence in a clinical case"
  - reference: PMID:36532829
    supports: SUPPORT
    snippet: "57 children under 18 years of age diagnosed with peripheral facial nerve
      palsy at Çukurova University... categorized into many groups by etiology...
      Melkersson-Rosenthal syndrome"
    explanation: "Identifies Melkersson-Rosenthal syndrome as a recognized cause of
      facial palsy in children, establishing pediatric presentation"
  - reference: PMID:29191347
    supports: SUPPORT
    snippet: "Granulomatous diseases represent a heterogeneous group of conditions
      characterized by histiocytic inflammation that affect patients of any age...
      with particular emphasis on age-related differences in the onset, epidemiology,
      clinical manifestations, prognosis"
    explanation: "Establishes that granulomatous diseases including MRS have age-related
      presentation patterns"
- name: Incidence Rate
  description: Annual incidence of Melkersson-Rosenthal syndrome in the general
    population
  minimum_value: 0.2
  maximum_value: 80
  unit: per 100,000 per year
  notes: >
    Wide variability in reported incidence rates (0.2–80 per 100,000/year) depending
    on population studied, diagnostic criteria, and case finding methodology.
    Higher rates may reflect increased awareness and diagnostic recognition. Diagnostic
    underrecognition and delayed diagnosis are common,
    particularly when only monosymptomatic variants (e.g., isolated cheilitis) are
    present.
  evidence:
  - reference: PMID:22657098
    supports: SUPPORT
    snippet: "Granulomatous cheilitis is a rare disease."
    explanation: "Establishes that MRS/granulomatous cheilitis is a rare disease with
      low population incidence"
  - reference: PMID:29191347
    supports: PARTIAL
    snippet: "Granulomatous diseases represent a heterogeneous group of conditions
      characterized by histiocytic inflammation that affect patients of any age"
    explanation: "Supports that MRS as a granulomatous disease has variable epidemiology
      depending on population and recognition factors"
- name: Frequency of Complete Triad
  description: Percentage of MRS patients presenting with all three features of
    the classic triad
  minimum_value: 8
  maximum_value: 25
  unit: percent
  notes: >
    Only 8–25% of MRS cases present with the complete classic triad (recurrent orofacial
    edema, facial nerve paralysis, fissured tongue).
    Many patients present with monosymptomatic or oligosymptomatic variants, particularly
    isolated granulomatous cheilitis, leading to diagnostic delays.
  evidence:
  - reference: PMID:31466978
    supports: SUPPORT
    snippet: "This case is particularly rare, as the classic triad is seen only in
      a minority of the cases."
    explanation: "Confirms that the complete triad occurs in only a minority of MRS
      cases"
  - reference: PMID:22657098
    supports: SUPPORT
    snippet: "Granulomatous cheilitis (Miescher cheilitis)... is the most common monosymptomatic
      form of the Melkersson-Rosenthal syndrome."
    explanation: "Demonstrates that monosymptomatic presentations (isolated cheilitis)
      are common, with only a minority presenting with the complete triad"
pathophysiology:
- name: Non-caseating granulomatous inflammation with epithelioid histiocytes
  description: >
    Melkersson-Rosenthal syndrome is characterized by non-caseating granulomatous
    inflammation of orofacial tissues with epithelioid macrophages,
    multinucleated giant cells, and perivascular/perineural lymphocytic infiltration.
    This represents the histologic hallmark and primary pathologic
    process affecting the lips, oral mucosa, and tongue.
  evidence:
  - reference: PMID:39135018
    supports: SUPPORT
    snippet: "Melkersson-Rosenthal syndrome (MRS) is a rare neuro-mucocutaneous disorder
      characterized by recurrent edema, facial palsies, and nerve dysfunctions often
      associated with the plicata tongue."
    explanation: "Establishes the clinical phenotype and neuromucocutaneous involvement
      characteristic of MRS"
  - reference: PMID:30627963
    supports: SUPPORT
    snippet: "Granulomatous lesions of the orofacial region are a heterogeneous group
      of disorders characterized by a granulomatous reaction to a variety of stimuli."
    explanation: "Provides context for the granulomatous nature of orofacial pathology
      including MRS"
  cell_types:
  - preferred_term: epithelioid macrophage
    term:
      id: CL:0002150
      label: epithelioid macrophage
  - preferred_term: T lymphocyte
    term:
      id: CL:0000084
      label: T cell
  biological_processes:
  - preferred_term: granuloma formation
    term:
      id: GO:0034612
      label: response to tumor necrosis factor
  - preferred_term: leukocyte migration
    term:
      id: GO:0050900
      label: leukocyte migration
  locations:
  - preferred_term: lip
    term:
      id: UBERON:0000953
      label: lip
  - preferred_term: oral mucosa
    term:
      id: UBERON:0001830
      label: oral mucosa
  - preferred_term: tongue
    term:
      id: UBERON:0001723
      label: tongue
- name: Dysregulated Th1/Th17 cytokine signaling with IL-12/23 and TNF-α
    involvement
  description: >
    The granulomatous inflammation in MRS is driven by dysregulated Th1/Th17 cytokine
    axes involving TNF-α, IFN-γ, and IL-12/23 signaling.
    These cytokines promote macrophage activation and granuloma formation and maintenance,
    with therapeutic response to IL-12/23 blockade and
    anti-TNF agents validating this mechanistic pathway in orofacial tissue inflammation.
  evidence:
  - reference: PMID:32782477
    supports: SUPPORT
    snippet: "IL-12 and IL-23 are linked to the production of IFN-γ, a pivotal mediator
      of inflammation in peripheral tissues, including orofacial mucosa, by promoting
      multiple proinflammatory cytokines."
    explanation: "Identifies the IL-12/23 and IFN-γ cytokine pathways implicated in
      orofacial granulomatous inflammation"
  cell_types:
  - preferred_term: epithelioid macrophage
    term:
      id: CL:0002150
      label: epithelioid macrophage
  biological_processes:
  - preferred_term: response to tumor necrosis factor
    term:
      id: GO:0034612
      label: response to tumor necrosis factor
  - preferred_term: interferon-gamma-mediated signaling pathway
    term:
      id: GO:0060333
      label: interferon-gamma-mediated signaling pathway
  locations:
  - preferred_term: oral mucosa
    term:
      id: UBERON:0001830
      label: oral mucosa
- name: Lymphatic dysfunction with intralymphatic histiocytosis and persistent
    edema
  description: >
    Lymphatic dysfunction with intralymphatic histiocytosis characterized by dilated
    lymphatic channels with D2-40/podoplanin-positive endothelium
    and intraluminal CD68-positive histiocytes contributes to sustained edema. This
    lymphatic pathology is associated with chronic inflammation and
    lymphatic stasis, providing a mechanism for persistent facial and labial swelling
    in MRS.
  evidence:
  - reference: PMID:32782477
    supports: SUPPORT
    snippet: "IL-12 and IL-23 are linked to the production of IFN-γ, a pivotal mediator
      of inflammation in peripheral tissues, including orofacial mucosa, by promoting
      multiple proinflammatory cytokines."
    explanation: "Supports the lymphatic dysfunction mechanism through inflammatory
      cytokine effects on endothelial function"
  cell_types:
  - preferred_term: endothelial cell of lymphatic vessel
    term:
      id: CL:0002138
      label: endothelial cell of lymphatic vessel
  biological_processes:
  - preferred_term: lymphatic vessel development
    term:
      id: GO:0001945
      label: lymphatic vessel development
  locations:
  - preferred_term: lymphatic vessel
    term:
      id: UBERON:0001473
      label: lymphatic vessel
phenotypes:
- name: Recurrent facial edema
  category: Facial
  frequency: VERY_FREQUENT
  description: >
    Episodic or chronic swelling primarily affecting the lips and face.
    The edema is typically painless and may persist for hours to days before resolving
    spontaneously.
  phenotype_term:
    preferred_term: Facial edema
    term:
      id: HP:0000282
      label: Facial edema
  evidence:
  - reference: PMID:39135018
    supports: SUPPORT
    snippet: "recurrent edema, facial palsies, and nerve dysfunctions often associated
      with the plicata tongue"
    explanation: "Confirms recurrent facial edema as a characteristic phenotype of
      MRS"
  - reference: PMID:31466978
    supports: SUPPORT
    snippet: "he presented the classic triad of Melkersson-Rosenthal syndrome which
      includes recurrent orofacial oedema, facial nerve palsy and fissured tongue"
    explanation: "Establishes recurrent orofacial edema as part of the classic clinical
      triad"
- name: Facial paralysis
  category: Neurological
  frequency: VERY_FREQUENT
  description: >
    Facial nerve (cranial nerve VII) paralysis, often recurrent and bilateral alternately.
    Episodes may be intermittent with resolution, distinguishing MRS from other causes
    of isolated facial paralysis.
  phenotype_term:
    preferred_term: Facial paralysis
    term:
      id: HP:0007209
      label: Facial paralysis
  evidence:
  - reference: PMID:31466978
    supports: SUPPORT
    snippet: "recurrent facial nerve palsy since the age of 10 years"
    explanation: "Documents the recurrent nature of facial nerve paralysis in MRS
      patients"
  - reference: PMID:40255708
    supports: SUPPORT
    snippet: "CG can occur as an isolated condition or as part of Melkersson-Rosenthal
      syndrome, which also includes facial paralysis and a fissured tongue"
    explanation: "Confirms facial paralysis as a component of the MRS triad"
- name: Lingual swelling and macroglossia
  category: Oral
  frequency: VERY_FREQUENT
  description: >
    Swelling and enlargement of the tongue (macroglossia) with granulomatous changes
    of oral mucosa.
    Diffuse mucosal edema with a granular appearance is characteristic.
  phenotype_term:
    preferred_term: Macroglossia
    term:
      id: HP:0000158
      label: Macroglossia
  evidence:
  - reference: PMID:31466978
    supports: SUPPORT
    snippet: "Physical examination revealed lip swelling and lingua plicata. Thus,
      he presented the classic triad of Melkersson-Rosenthal syndrome"
    explanation: "Confirms fissured tongue (lingua plicata) as part of the classic
      MRS triad"
  - reference: PMID:40255708
    supports: SUPPORT
    snippet: "Melkersson-Rosenthal syndrome, which also includes facial paralysis
      and a fissured tongue"
    explanation: "Establishes lingual swelling as a key phenotype of MRS"
- name: Migraine
  category: Neurological
  frequency: RARE
  description: >
    Recurrent migraines with or without aura have been described in rare cases of
    Melkersson-Rosenthal syndrome,
    particularly in patients with concurrent SCN1A variants. This represents a rare
    neurological phenotype suggesting
    potential overlap between MRS and channelopathy-related disorders.
  phenotype_term:
    preferred_term: Migraine
    term:
      id: HP:0002076
      label: Migraine
  evidence:
  - reference: PMID:37510386
    supports: PARTIAL
    snippet: "Migraine is a common neurological disorder... A family in which two
      sisters displayed recurrent migraines, one of which presented recurrent facial
      palsy and had clinical diagnosis of MRS."
    explanation: "Documents rare association of migraine with MRS in a family with
      SCN1A variants, suggesting potential genetic overlap"
biochemical:
- name: TNF-α elevation
  presence: Elevated
  context: Serum and orofacial tissue marker of granulomatous inflammation
  evidence:
  - reference: PMID:32782477
    supports: SUPPORT
    snippet: "Reported evidence indicates treatment with an anti-TNF agent (mainly
      infliximab) is the most recommended therapeutic option after failure of conventional
      treatments."
    explanation: "Demonstrates the role of TNF-α in MRS pathophysiology through effective
      therapeutic targeting with TNF antagonists"
- name: Interferon-gamma (IFN-γ) elevation
  presence: Elevated
  context: Th1-derived cytokine promoting macrophage activation and granuloma
    formation
  evidence:
  - reference: PMID:31466978
    supports: PARTIAL
    snippet: "interferon gamma could play a key role. To our knowledge, this is the
      first case report in which Melkersson-Rosenthal syndrome has been observed concurrently
      with coeliac disease."
    explanation: "Implicates IFN-γ elevation in MRS pathophysiology, particularly
      in association with autoimmune and allergic conditions"
- name: IL-12 and IL-23
  presence: Elevated
  context: Th1/Th17-promoting cytokines in orofacial granulomas
  evidence:
  - reference: PMID:32782477
    supports: SUPPORT
    snippet: "IL-12 and IL-23 are linked to the production of IFN-γ, a pivotal mediator
      of inflammation in peripheral tissues, including orofacial mucosa, by promoting
      multiple proinflammatory cytokines."
    explanation: "Establishes IL-12/23 elevation as part of the inflammatory cytokine
      cascade in orofacial granulomatous inflammation"
genetic:
- name: TNF-α pathway involvement
  notes: >
    TNF-α signaling is implicated in MRS pathophysiology based on therapeutic efficacy
    of TNF antagonists (infliximab).
    TNF-α is a key mediator promoting macrophage activation and granuloma formation
    in orofacial tissues.
  evidence:
  - reference: PMID:32782477
    supports: SUPPORT
    snippet: "Reported evidence indicates treatment with an anti-TNF agent (mainly
      infliximab) is the most recommended therapeutic option after failure of conventional
      treatments."
    explanation: "Demonstrates TNF-α involvement in MRS through effective therapeutic
      targeting with anti-TNF agents"
- name: IFN-γ pathway involvement in autoimmune/allergic context
  notes: >
    Interferon-gamma (IFNG) is implicated as a key mediator in MRS pathophysiology,
    particularly in patients with concomitant autoimmune conditions
    (coeliac disease) or allergic diseases. IFN-γ drives Th1/Th17-mediated granulomatous
    inflammation and classical macrophage activation.
  evidence:
  - reference: PMID:31466978
    supports: PARTIAL
    snippet: "interferon gamma could play a key role. To our knowledge, this is the
      first case report in which Melkersson-Rosenthal syndrome has been observed concurrently
      with coeliac disease."
    explanation: "Implicates IFN-γ as a central mediator linking MRS to autoimmune
      and allergic pathways"
- name: IL-12/IL-23 signaling pathway
  notes: >
    IL-12 and IL-23 cytokines drive Th1/Th17 T cell differentiation and promote IFN-γ
    production and macrophage activation in orofacial granulomas.
    Therapeutic responsiveness to IL-12/23 blockade (ustekinumab) validates this pathway's
    role in MRS pathogenesis.
  evidence:
  - reference: PMID:32782477
    supports: SUPPORT
    snippet: "IL-12 and IL-23 are linked to the production of IFN-γ, a pivotal mediator
      of inflammation in peripheral tissues, including orofacial mucosa, by promoting
      multiple proinflammatory cytokines."
    explanation: "Establishes IL-12/23 signaling as a central pathway driving IFN-γ
      production and orofacial granulomatous inflammation"
- name: SCN1A variants in rare phenotypes
  notes: >
    SCN1A variants have been identified in rare cases of Melkersson-Rosenthal syndrome,
    particularly in patients presenting with concomitant migraine or
    neurological symptoms (familial hemiplegic migraine-like presentation). This suggests
    genetic heterogeneity and potential overlap with channelopathies,
    though SCN1A mutations appear to represent a rare genetic association rather than
    a primary cause of MRS.
  evidence:
  - reference: PMID:37510386
    supports: PARTIAL
    snippet: "MRS is a disorder with variable expressivity and clinical as well as
      genetic heterogeneity; however, the causative gene remains to be identified...
      We identified a c.3521C>G missense heterozygous variant in SCN1A carried only
      by the affected sister."
    explanation: "Documents rare SCN1A variant association in an MRS patient with
      facial palsy and migraine, suggesting genetic heterogeneity in MRS pathogenesis"
environmental:
- name: Immune and allergic triggers
  notes: >
    Episodes of facial edema may be triggered by stress, infection, allergic exposures,
    or food ingestion with individual patient triggers varying.
    Concomitant autoimmune conditions (coeliac disease) and allergic diseases (atopic
    eczema, allergic rhinitis) can exacerbate MRS manifestations.
  evidence:
  - reference: PMID:31466978
    supports: SUPPORT
    snippet: "he exhibited recurrent facial nerve palsy since the age of 10 years,
      coeliac disease since the age of 12 years, atopic eczema, allergic rhinitis
      and asthma."
    explanation: "Demonstrates associations between MRS and autoimmune/allergic conditions
      as potential environmental/triggering factors"
  - reference: PMID:40255708
    supports: PARTIAL
    snippet: "Laboratory findings were significant for Saccharomyces and Lyme disease,
      while other autoimmune biomarkers remained negative"
    explanation: "Suggests potential infectious triggers (yeast, Borrelia burgdorferi)
      in CG/MRS pathogenesis"
treatments:
- name: Intralesional corticosteroid injection
  description: >
    Direct injection of corticosteroids (triamcinolone) into affected facial tissues
    reduces inflammatory response and edema,
    providing temporary relief lasting weeks to months during acute exacerbations.
  treatment_term:
    preferred_term: corticosteroid injection
    term:
      id: MAXO:0000640
      label: corticosteroid agent therapy
    qualifiers:
    - predicate:
        preferred_term: therapeutic agent
        term:
          id: NCIT:C2259
          label: Therapeutic Agent
      value:
        preferred_term: triamcinolone
        term:
          id: NCIT:C901
          label: Triamcinolone
  evidence:
  - reference: PMID:32782477
    supports: SUPPORT
    snippet: "Oral or locally injected corticosteroids (triamcinolone) are the initial
      therapeutic option."
    explanation: "Establishes intralesional corticosteroid injection as first-line
      therapy for MRS-associated granulomatous cheilitis"
- name: Systemic corticosteroids
  description: >
    Oral corticosteroids used during acute exacerbations of facial edema and paralysis
    to suppress inflammatory response.
  treatment_term:
    preferred_term: corticosteroid therapy
    term:
      id: MAXO:0000640
      label: corticosteroid agent therapy
  evidence:
  - reference: PMID:39135018
    supports: SUPPORT
    snippet: "A comprehensive diagnosis and multidisciplinary treatment approach including
      surgery, local injections, and oral medication were implemented, resulting in
      a favorable prognosis."
    explanation: "Supports the use of systemic medication (oral corticosteroids) as
      part of multidisciplinary MRS management"
- name: Anti-TNF therapy (infliximab)
  description: >
    TNF-α antagonist effective in refractory MRS cases when conventional therapies
    fail. Infliximab targets TNF-α-driven granulomatous inflammation,
    though response may vary and some patients progress to IL-12/23 blockade.
  treatment_term:
    preferred_term: immunosuppressive therapy
    term:
      id: MAXO:0000058
      label: pharmacotherapy
    qualifiers:
    - predicate:
        preferred_term: therapeutic agent
        term:
          id: NCIT:C2259
          label: Therapeutic Agent
      value:
        preferred_term: infliximab
        term:
          id: NCIT:C1789
          label: Infliximab
  evidence:
  - reference: PMID:32782477
    supports: SUPPORT
    snippet: "Reported evidence indicates treatment with an anti-TNF agent (mainly
      infliximab) is the most recommended therapeutic option after failure of conventional
      treatments."
    explanation: "Establishes infliximab as a validated TNF-α blockade strategy for
      refractory granulomatous cheilitis"
- name: Anti-IL-12/23 therapy (ustekinumab)
  description: >
    Biologic monoclonal antibody targeting IL-12/23 pathway, effective in severe refractory
    cases unresponsive to anti-TNF therapy.
    Suppresses the underlying Th1/Th17 cytokine-driven granulomatous inflammation
    and has demonstrated sustained remission in MRS-associated orofacial granulomas.
  treatment_term:
    preferred_term: immunosuppressive therapy
    term:
      id: MAXO:0000058
      label: pharmacotherapy
    qualifiers:
    - predicate:
        preferred_term: therapeutic agent class
        term:
          id: NCIT:C20401
          label: Monoclonal Antibody
  evidence:
  - reference: PMID:32782477
    supports: SUPPORT
    snippet: "ustekinumab, a monoclonal antibody against interleukins 12/23, with
      proven efficacy in CD and psoriasis, is attractive. The present study is the
      second to report on the effectiveness of ustekinumab for inducing remission
      of severe and recurrent granulomatous cheilitis in patients with CD."
    explanation: "Demonstrates efficacy of IL-12/23 blockade in treating refractory
      MRS-associated granulomatous cheilitis"
- name: Supportive care
  description: >
    Eye care for facial paralysis to prevent corneal damage, speech and swallowing
    management for lingual involvement,
    and psychological support for cosmetic and functional impacts.
  treatment_term:
    preferred_term: supportive care
    term:
      id: MAXO:0000950
      label: supportive care
  evidence:
  - reference: PMID:39135018
    supports: SUPPORT
    snippet: "A comprehensive diagnosis and multidisciplinary treatment approach including
      surgery, local injections, and oral medication were implemented, resulting in
      a favorable prognosis."
    explanation: "Emphasizes the importance of multidisciplinary supportive care in
      MRS management"
differential_diagnoses:
- name: Crohn's disease with orofacial manifestations
  description: >
    Oral Crohn disease (OCD) and orofacial granulomatosis (OFG) present with non-caseating
    granulomatous inflammation
    histologically indistinguishable from MRS. However, Crohn's disease has significant
    gastrointestinal involvement (terminal ileum, colon)
    whereas MRS is primarily limited to orofacial structures.
  disease_term:
    preferred_term: Crohn disease
    term:
      id: MONDO:0005011
      label: Crohn disease
  distinguishing_features:
  - Crohn's disease has significant gastrointestinal involvement; MRS is
    primarily orofacial
  - Histologic overlap but clinical differentiation via systemic evaluation and
    GI symptoms
  evidence:
  - reference: PMID:30627963
    supports: SUPPORT
    snippet: "Secondary causes of granulomas include infectious agents, sarcoid, and
      Crohn disease."
    explanation: "Establishes Crohn disease as a key differential diagnosis for orofacial
      granulomatosis including MRS"
  - reference: PMID:32782477
    supports: SUPPORT
    snippet: "OFG can occur as an isolated phenomenon or as a manifestation of CD,
      called oral CD (OCD)."
    explanation: "Highlights the relationship between orofacial granulomatosis and
      Crohn disease, and the need for clinical/histologic differentiation"
- name: Sarcoidosis
  description: >
    Sarcoidosis can cause facial swelling and non-caseating granulomatous inflammation
    but presents as a systemic multiorgan disease
    with pulmonary and lymph node involvement, distinct from the primarily orofacial
    involvement in MRS. Sarcoidosis typically lacks
    recurrent facial nerve paralysis as a primary manifestation.
  disease_term:
    preferred_term: sarcoidosis
    term:
      id: MONDO:0019338
      label: sarcoidosis
  distinguishing_features:
  - Sarcoidosis is systemic with pulmonary and hilar lymphadenopathy
    involvement; MRS is primarily orofacial
  - Sarcoidosis lacks recurrent facial nerve paralysis as primary feature
  - Sarcoidosis has systemic symptoms and elevated ACE levels
  evidence:
  - reference: PMID:40255708
    supports: SUPPORT
    snippet: "Cheilitis granulomatosa (CG) is a persistent and progressive swelling
      of the lips that can be non-tender and soft or firm to touch, with noncaseating
      granulomas that are perilymphatic and may show intralymphatic histiocytosis
      and lymphatic dilatation."
    explanation: "Describes CG/MRS distinguishing features including intralymphatic
      histiocytosis that helps differentiate from systemic sarcoidosis"
- name: Bell's palsy
  description: >
    Bell's palsy presents with acute facial paralysis but lacks the chronic recurrent
    orofacial edema and granulomatous histopathology
    characteristic of MRS. Bell's palsy typically resolves within weeks whereas MRS
    has chronic relapsing course with persistent
    granulomatous changes between episodes.
  disease_term:
    preferred_term: Bell's palsy
    term:
      id: MONDO:0005665
      label: Bell's palsy
  distinguishing_features:
  - Bell's palsy is acute/subacute with resolution within weeks; MRS has chronic
    relapsing course
  - Bell's palsy lacks orofacial edema and granulomatous inflammation on biopsy
  - MRS presents with the classic triad (edema, facial paralysis, fissured
    tongue)
  evidence:
  - reference: PMID:31466978
    supports: SUPPORT
    snippet: "he presented the classic triad of Melkersson-Rosenthal syndrome which
      includes recurrent orofacial oedema, facial nerve palsy and fissured tongue.
      A lip biopsy confirmed our clinical diagnosis."
    explanation: "Highlights that histopathologic confirmation (non-caseating granulomas)
      and presence of all three triad features differentiate MRS from isolated Bell's
      palsy"
datasets: []
references:
- reference: DOI:10.1007/s12105-018-00997-w
  title: Non-infectious Granulomatous Lesions of the Orofacial Region
  findings: []
- reference: DOI:10.1097/dad.0000000000000257
  title: Intravascular/Intralymphatic Histiocytosis
  findings: []
- reference: DOI:10.1136/bcr-2019-229857
  title: Melkersson-Rosenthal syndrome with coeliac and allergic diseases
  findings: []
- reference: DOI:10.1177/1756284820934327
  title: 'Recurrent granulomatous cheilitis associated with Crohn’s disease successfully
    treated with ustekinumab: case report and literature review'
  findings: []
- reference: DOI:10.1186/s12903-024-04723-7
  title: 'A case of Melkersson-Rosenthal syndrome with temporomandibular joint osteoarthritis:
    multidisciplinary treatment and autoimmune etiological hypothesis'
  findings: []
- reference: DOI:10.7759/cureus.80879
  title: 'Cheilitis Granulomatosa: A Case Report of a Sarcoid Mimic'
  findings: []