A rare case of Systemic Lupus Erythematosus with Congenital Bifid Tongue

Systemic Lupus Erythematosus (SLE) is an autoimmune disorder with multiple aetiology, presents with varied cutaneous manifestations and systemic involvement, primarily affects women in her child bearing age. Congenital bifid tongue is an anomaly due to nonfusion of the two lingual swellings. It can occur either as an isolated entity or in association with syndromes like Opitz G syndrome, Oral-Facial-Digital syndrome type I, Klippel Feil anomaly and Larsen syndrome or even as very rare feature in infants born to diabetic mothers. We present a case report of 23 year old female, a second child from a nonconsanguineous marriage with no significant antenatal or postnatal history, diagnosed of SLE. She had secondary amenorrhoea and hypothyroidism, examination revealed reduced secondary sexual characteristics, short stature, orbital hypertelorism and a bifid tongue which was present since birth. Recent studies suggest there is increased risk of autoimmune diseases particularly SLE development via the gene transmitted by X chromosome. It is also found that SLE patients are commonly associated with X chromosome polysomy. This case is unusual as the patient had multiple features suggesting genetic involvement, both SLE and bifid tongue is individually found to have genetic roots and till date there is no single case of SLE with a congenital bifid tongue in literature.