January/February - West Virginia State Medical Association

| Scientific Article
Moreover, periorbital and upper
eyelid edema have been reported
in multiple cases (14-18). Recurrent
facial nerve palsy is reported in
47% of patients, while lingua
plicata is reported in up to 50% (5).
Furthermore, in many cases various
additional neurological symptoms
have been reported, including ear
pain, diplopia, vertigo, tinnitus,
and decreased saliva (4). In one
study 11% of patients reported
associated migraines, which our
patient also experienced (5).
Histologically, the early
stage is often represented by
edema, lymphangiectasia, and
a predominately perivascular
lymphoplasmacytic infiltrate (5,19).
In the later stages, after weeks
of persistent edema, the classic
histopathologic picture emerges.
The classic histopathologic findings
are characterized by small and
scattered noncaseating epithelioid
granulomas with lymphangitis
and perilymphangitis (5,19).
Granulomatous inflammation is
not present in all biopsies, and
its absence does not exclude
the diagnosis of MRS.
The differential diagnosis of
persistent swelling of the lip is
extensive (Table 1), but a thorough
history and careful clinical
Table 1. Differential diagnosis of
persisent swelling of the lip (5,11).
Allergic contact dermatitis
Ascher syndrome
Cheilitis glandularis
Cheilitis granulomatosa
(Melkersson-Rosenthal syndrome)
Crohn’s disease
Facial edema with eosinophilia
Granulomatous infection
Hemangioma
Hereditary angioedema
Lymphangioma
Neoplastic
Odontogenic infection
Recurrent erysipelas
Sarcoidosis
examination will usually eliminate
many diagnostic possibilities.
Although many of the causes
of recurrent lip swelling can be
excluded on the basis of history and
physical examination, a full-thickness
mucosal biopsy is needed in order to
make a definitive diagnosis. Special
stains of the specimen are used in an
effort to rule out a mycobacterial or
deep fungal infection; polarization
can help identify a foreign material.
Correlation of historical and clinical
data with the histologic findings
ultimately culminates in the diagnosis.
MRS is often refractory to
treatment, with no individual or
combination therapy resulting
in complete remission. Topical,
intralesional and systemic
corticosteroids have been used
with some success. Other reported
therapies, including prednisone,
methotrexate, hydroxychloroquine,
dapsone, thalidomide, ranitidine,
diphenhydramine, metronidazole,
clofazimine, sulfasalazine, penicillin,
erythromycin, clindamycin,
tetracycline, and minocycline as
single therapy or as a combination
regimen, have been used with
some efficacy (2,15,20). Recently,
Barry et al. reported a case of
granulomatous cheilits refractory to
minocycline, erythromycin, topical
tacrolimus, and clofazimine and was
successfully treated with infliximab
(21). Radiotherapy, facial nerve
decompression for the facial palsy,
and surgical debulking for cosmesis
have also been used (15,22,23).
References
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Please contact the authors for additional references.
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