Case Report 1 - Female child with "coup de sabre"
Age of onset: 3 years
Initial Symptoms: Indentation and bluish-colored line formed down the right side of forehead ("coup de sabre" lesion) with loss of hair approximately the size of a quarter above this area. Thinning of eyelashes and eyebrow on right side. Right eye was non-reactive to light and had developed uveitis(inflammation). By age 4, child experienced a seizure, which caused her right arm to shake uncontrollably. By age 6, soft tissues of head and face on right side showed significant atrophy.
Disease continued to progress throughout childhood and adolescence.
By Age 18: Uveitis/inflammation and decreasing vision of right eye continues; continued, progressive atrophy of soft tissues of right side of face, head and right eye, which has resulted in right part of face and right eye pushed back. Face is now asymmetrical. Continues to lose hair above right part of head and increased loss of eyelashes and eyebrow on affected right side. Left side of face and left eye are normal. No other body part is affected.
Medications tried: Cellcept, prednisone, methotrexate injections, acyclovir and Pred-forte (eye drops).
Parent reports that daughter consults every 6 months with a Pediatric Rheumatologist and ophthalmologist.
