Melkersson Rosenthal syndrome
Balasubramanian M.S. D.L.O.
Rosenthal syndrome is a rare, non-caseating granulomatous disease
which is characterised by a triad which includes facial paralysis,
orofacial oedema and lingua plicata (scrotal tongue, fissured tongue,
or furrowed tongue). It should be stressed that these triad of
symptoms is not frequently seen in its complete form. The diagnosis
of this condition is rather difficult because the classic triad of
symptoms are seen only in 10% of patients with Melkersson –
this disease remains largely unknown however the following factors
have been hypothetically implicated:
histopathologic features of this condition include:
Non caseating epitheloid cell granulomas
Presence of multinulceated Langhan's type giant cells
Presence of perivascular mononuclear inflammatory cell
Presence of perivascular fibrosis
Melkersson Rosenthal syndrome may demonstrate the classic triad
simultaneously or at different times. It should be stressed that in
a patient with orofacial oedema the presence of one of the features
given below is sufficient to make a diagnosis of Melkersson Rosenthal
Idiopathic facial palsy
be one of the dominant signs of Melkersson Rosenthal syndrome. The
features of facial oedema in these patients include:
Non pitting odema
Commonly affecting upper lip
Facial oedema may last from hours to weeks and may also recur
oedema should be differentiated from angioneurotic oedema by its
persistent nature, and non responsiveness to antihistamins and. Oedema
is caused by fibrosis around blood vessels causing
extravasation of fluid from them. The fibrosis also prevents
reabsorption of extravasated fluid.
patients facial paralysis may occur months to years before or after
the onset of facial oedema. Facial palsy is commonly LMN type,
unilateral / bilateral, partial or complete. 90% of these patients
had recovery of facial nerve function.
as scrotal tongue / fissured tongue is commonly considered to be
congenital developmental malformation.
evaluation which includes CT / MRI/Chest x-rays are non contributary
in these patients. Histopathological features are the only available
confirmatory diagnostic evaluation.
associated with Melkersson Rosenthal syndrome:
intralesional steroid therapy.
been tried with varying degrees of success include:
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