Patient characteristics
| 41 year old man |
| Periodic fever and a generalised maculopapular itching erythematous rash since the age of about 8 months |
| Arthralgia and arthritis with transient swelling beginning in the first year of life |
| Perceptive deafness beginning after about 30 years |
| Suffering from abdominal pain and persistent haemorrhagic diarrhoea with increasing age |
| No increased levels of autoimmune antibodies |
| Skin histology: cellular infiltrate mainly containing neutrophils |
| Cranial MRI: internal hydrocephalus and empty sella |
| Lumbar puncture: sterile chronic meningitis |
| No growth or mental retardation |