International Journal of Clinical Case Report, 2016, Vol.6, No.16, 1-4
3
Figure 4 Incisional biopsy done on lower lip
Figure 5 Histopathological examination revealing aggregates of non-caseating epitheloid cell granulomas with multinucleated,
Langerhan’s giant cells and chronic inflammatory cell infiltrates comprising chiefly of lymphocytes
2 Discussion
Melkersson-Rosenthal syndrome (MRS) is a rare neuro-muco-cutaneous disorder presenting with the classic triad
of Miescher’s cheilitis granulomatosa (CG), facial palsy and lingua plicata (Gürkan et al., 2015; Agarwal et al.,
2011; Talabi O.A., 2011). The exact etiology and pathogenesis of this syndrome is still unclear, however, certain
factors such as herpes simplex infection, Epstein Barr virus infection, cytomegalovirus infection, bacterial
infection, allergic reaction, immune-mediated phenomena, genetic factors etc., which have been considered
etiological but not yet proven (Ozgursoy et al., 2011; Hathiram et al., 2000). Some studies reported an autosomal
dominant inheritance pattern with the responsible gene mapping to chromosome 9 pH (Rose et al., 2011). In
majority of the MRS patients, most common finding is lip edema. In about 40% of cases, cheilitis granulomatosa
is the presenting sign of MRS, which consequently progresses to neurologic signs (Vibhute et al., 2013). It may
involve upper or lower lip or both the lips (Critchlow and Chang, 2014). In our case, both the lips were involved.
The diagnosis of cheilitis granulomatosa is made by histopathologic findings of non-caseating granulomas. The
second most common sign of MRS which is seen rarely is the facial palsy. Facial palsy may be partial or complete
and may be unilateral or bilateral. It may be due to the compression of nerve by tissue edema or may be due to
granulomatous infiltration of the nerve and nerve sheath (Agarwal et al., 2011). The advanced radiological
investigations such as computed tomography and magnetic resonance imaging are advised to rule-out any
underlying pathology causing facial nerve paralysis. Facial nerve paralysis is seen in 10% to 20% of the cases
reported (Vibhute et al., 2013). The third uncommon sign is fissured or furrowed tongue or lingua plicata. It is
most commonly seen as a congenital anomaly in general population. But in our case, classic triad MRS was
present, which is rarely seen. It is most commonly seen in 2nd and 3rd decade of life. Only 30 cases of childhood
