J Med Cases

Journal of Medical Cases, ISSN 1923-4155 print, 1923-4163 online, Open Access

Article copyright, the authors; Journal compilation copyright, J Med Cases and Elmer Press Inc

Journal website http://www.journalmc.org

Case Report

Volume 9, Number 9, September 2018, pages 303-308

Melkersson-Rosenthal Syndrome Still a Mystery: A Case Report and Suggested Guideline Investigations

Figure 1. (a, b) Right side edema with swollen upper lip. (c, d) Fissured tongue, gingival hypertrophy. (e, f) Facial nerve examination; flattened right forehead, weakness in closing the right eye. (g, h) CT scan showing right side facial swelling and upper lip swelling.

**Table 1.** Investigations
Investigation^a	Results
ACE: angiotensin-converting antibody; C3, C4: third and fourth components of complement; HIV: human immunodeficiency virus; RAST: radioallergosorbent test; CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; TG: thyroglobulin; TPO: thyroid peroxidase; TTG: tissue transglutaminase; CCP: cyclic citrullinated peptide; ANCA: anti-neutrophilic cytoplasmic antibody; PPD: purified protein derivative. ^aPlanned to be performed if symptoms of facial palsy persisted, but the patient missed the follow-up appointment: C1 esterase inhibitor, ANCA, immunoglobulin E, total iron profile, chest plain radiography, PPD (for tuberculosis), hepatitis C, serum cryoglobulins, patch test, head magnetic resonance imaging.
Complete blood count	Normal
CRP	Mildly elevated: 12.5 mg/L (normal: 0 - 3.0) Normalized after steroid course (1.3 mg/L)
ESR	Normal
Thyroid function tests	Normal
Anti-TG antibodies, anti-TPO antibodies	Negative
Renal function tests	Normal
TTG, anti-gliadin antibody	Negative
Anti-CCP	Negative
C3	Normal
C4	Mildly decreased. 0.13 g/L (normal 0.17 - 0.52)
Antinuclear antibody	Negative
HIV	Negative
RAST for chicken meat, mutton, and beef	Negative
ACE level	Normal
Rheumatoid factor	Negative

Table 1. Investigations

Investigation^a

Results

ACE: angiotensin-converting antibody; C3, C4: third and fourth components of complement; HIV: human immunodeficiency virus; RAST: radioallergosorbent test; CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; TG: thyroglobulin; TPO: thyroid peroxidase; TTG: tissue transglutaminase; CCP: cyclic citrullinated peptide; ANCA: anti-neutrophilic cytoplasmic antibody; PPD: purified protein derivative. ^aPlanned to be performed if symptoms of facial palsy persisted, but the patient missed the follow-up appointment: C1 esterase inhibitor, ANCA, immunoglobulin E, total iron profile, chest plain radiography, PPD (for tuberculosis), hepatitis C, serum cryoglobulins, patch test, head magnetic resonance imaging.

Complete blood count

Normal

CRP

Mildly elevated: 12.5 mg/L (normal: 0 - 3.0)
Normalized after steroid course (1.3 mg/L)

ESR

Normal

Thyroid function tests

Normal

Anti-TG antibodies, anti-TPO antibodies

Negative

Renal function tests

Normal

TTG, anti-gliadin antibody

Negative

Anti-CCP

Negative

Normal

Mildly decreased. 0.13 g/L (normal 0.17 - 0.52)

Antinuclear antibody

Negative

HIV

Negative

RAST for chicken meat, mutton, and beef

Negative

ACE level

Normal

Rheumatoid factor

Negative

**Table 2.** Suggested Guideline Investigations
ACE: angiotensin-converting antibody; C3, C4: third and fourth components of complement; RAST: radioallergosorbent test; CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; TTG: tissue transglutaminase; ANCA: anti-neutrophilic cytoplasmic antibody; PPD: purified protein derivative.
CBC, ESR, CRP
Renal and liver function tests
Total immunoglobulins, subgroups
Thyroid function tests, thyroid antibodies
Anti-gliadin, TTG
C3, C4 levels
Antinuclear antibody, ANCA, renal function tests
C1 esterase inhibitor
ACE level
Chest plain radiography, PPD (for tuberculosis)
Biopsy to determine histopathology and to guide screening for microbial agents
Detailed history, including the first symptom of the triad to appear, number of years having the disease, when the biopsy was done, treatment used, number of relapses

Table 2. Suggested Guideline Investigations

ACE: angiotensin-converting antibody; C3, C4: third and fourth components of complement; RAST: radioallergosorbent test; CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; TTG: tissue transglutaminase; ANCA: anti-neutrophilic cytoplasmic antibody; PPD: purified protein derivative.

CBC, ESR, CRP

Renal and liver function tests

Total immunoglobulins, subgroups

Thyroid function tests, thyroid antibodies

Anti-gliadin, TTG

C3, C4 levels

Antinuclear antibody, ANCA, renal function tests

C1 esterase inhibitor

ACE level

Chest plain radiography, PPD (for tuberculosis)

Biopsy to determine histopathology and to guide screening for microbial agents

Detailed history, including the first symptom of the triad to appear, number of years having the disease, when the biopsy was done, treatment used, number of relapses