Melkersson-Rosenthal Syndrome: a rare familial case with granulomatous cheilitis and fissured tongue in both father and son

Melkersson-Rosenthal Syndrome (MRS) is a rare condition with an unknown aetiopathogenesis. Infectious, genetic and inflammatory aetiologies are implicated in MRS, but familial cases of MRS are exceedingly rare. There are several case series in literature where MRS was associated with rosacea. Recurrent facial nerve palsy, recurrent facial swelling and fissured tongue constitute the classic triad in MRS. Diagnosis is often difficult as the classic triad of features are present in only 25-30% of cases. We report the rare occurrence of MRS in both father and son. Index patient who is a23 year old male had concurrent rosacea also treated with doxycycline has resulted in improvement of both facial swelling and rosacea. He has had a history of facial nerve palsy three years back, followed by progressive swelling of lips and tongue. Swelling of lips and tongue was initially fluctuating and later became persistent. Patient also developed mild photosensitivity, persistent erythema over the nose and conjunctival injection for one year duration. Skin biopsy revealed granuloma, and, slit skin smears from the lips were negative for acid fast bacilli. The diagnosis of granulomatous cheilitis secondary to MRS with concurrent rosacea was made and was treated successfully.