Melkersson-Rosenthal syndrome is a systemic
neurological granulomatous disease which is relatively
rare and has an unknown etiology. The reported
incidence of MRS is 0.08%, and it is usually seen in
adolescence.
7 However, most studies show an older
distribution in early adulthood with a 2:1 female preponderance.
10 The pathogenesis of the disease
is still unknown; however, there are several factors
which are thought to play a role in the disease process,
for example heredity, chronic infection, allergies,
and hypersensitivity to bacteria.
11 Although both
genetic and acquired factors have been implicated, the
exact etiology of MRS remains unclear. It has been
suggested that genetic predisposition is involved in the
pathogenesis of the disease because some cases have
occurred within the same family.
12 Subsequently, a
gene defect at chromosome 9 p11 has been discovered
to have a relationship with MRS, so evidence exists
for autosomal dominant inheritance. On the other
hand, genetic factors do not account for all cases, and
infections (viral and bacterial), autoimmunity, and
hypersensitivity all have been implicated as etiologic
factors with reasonable evidence.
12
While there are three features that form the classic
triad of MRS, they are rarely encountered at the time
of presentation, making the diagnosis challenging.12,13
Our case had all three features of the disease: recurrent
peripheral facial palsy, a furrowed tongue, and orofacial
edema.12 Additionally, she had a comorbidity with SLE.
Systemic lupus erythematosus is a common,
chronic, inflammatory autoimmune disease that it
is diagnosed based on the American College of
Rheumatology (ACR) criteria. Our patient had been
diagnosed with SLE according to these criteria
five years prior to her arrival at our clinic.14 As
we learned from her medical records, she had
complaints of fatigue, fever, and weight loss, and
her initial examination had revealed a malar rash,
photosensitivity, and oral aphthous ulcers with an
abnormal antibody to the native DNA (Anti-ds-DNA)
titer. In a recent examination, our patient had
photosensitivity, oral aphthous ulcers, and serological
findings such as positive ANA, anti dsDNA, and
hypocomplementemia which made us consider the
attack as lupus activation.
However, a review of literature showed that
bilateral facial paralysis may be present in the course
of lupus activation.4,15 As we learned from the patient
herself (later confirmed by her medical records), none
of the facial paralysis attacks indicated a temporal
relationship with the activation of lupus. Moreover,
there were no clinical, laboratory, or serological
findings suggesting lupus activation in her most
recent facial paralysis attack. After examining all the
facts, the patient's facial paralysis attacks were then
attributed to MRS.
A few reports exist which show comorbidity of
DLE with MRS,7-9 but there are no reports indicating
a correlation between SLE and MRS. There is limited
literature attesting to the fact that there may be a
genetic predisposition for MRS or that it can present
as a symptom of Crohn's disease or sarcoidosis,13
but there is no information about how the disease
interferes with SLE.
Melkersson-Rosenthal syndrome is characterized
by a triad of symptoms: orofacial edema, peripheral
facial paralysis, and lingua plicata, but all three
features of the disease are rarely encountered at
the time of presentation, making diagnosis more
difficult.4,13
Angioedema, acute allergic reaction, and parotitis
should be considered in the differential diagnosis,2
but in our case, the IgE levels and amylase levels
were normal, and the patient's history and physical
examination revealed no correlation with these
disorders.
We presented this rare case of MRS with a complete
triad in order to report the comorbidity of lupus with
the disease, an uncommon occurrence in the literature,
Further research on MRS is needed to provide more
data on this uncommon disease.
Declaration of conflicting interests
The authors declared no conflicts of interest with
respect to the authorship and/or publication of this
article.
Funding
The authors received no financial support for the
research and/or authorship of this article.
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