- Triangular face (pointed mandible).
- Atypical ears: prominent “winged”; small, round and without lobule; lobule attached to the face; Different shapes: kidney shape, “Dumbo” ears, pointed “Mr. Spock” like ears, soft ears, bent helix ears; question mark shape ears; operated ears; etc.
- Atypical nose: nodule between the bone and the cartilage; deviated nasal septum; operated nose.
- Light blue sclera, more noticeable in women.
- Antimongoloid slan
Note: The JHS typical facial appearance was first described in the literature by Jaime F. Bravo, MD, both in Reumatologia 2004;20(1):24-30, and Arthritis Rheum 2006;54:515-23.