Dominant disease associating

Synopsis

 systemic granulomatosis

• cutaneous granulomatosis
  • noncaseating granulomatous skin infiltration
  • ichtyosis

• granulomatous synovitis
• cranial basis granulomatosis
  • cranial neuropathies
  • corticosteroid-responsive hearing loss

 nongranulomatous uveitis
 boutonniere deformities
 flexion contractures of fingers and toes
 cysts over wrist and ankle joints
 intermittent generalized erythematous papular rash

Etiology

 germline mutations of the CARD15 (also involved in susceptibility to Crohn disease)

See also

 systemic granulomatoses