Concurrence of Incontinentia Pigmenti and Behçet's Disease.

We report here a rare case of incontinentia pigmenti (IP) in a 10-year-old girl who developed Behçet's disease. IP was diagnosed in infancy and Behçet's disease was diagnosed at 10 years of age. The initial presentations of Behçet's disease were spiking fever and recurrent painful oral and genital ulcers that were refractory to antibiotics. After corticosteroid treatment, her fever subsided and ulcers subsequently healed. The patient's mother and sister were also diagnosed with IP. Her mother had suffered from Behçet's disease since her teenage years and it was complicated with colon perforation. Although there are several reports on the combination of IP and Behçet's disease, this is the first reported case of a family with such concurrence.