2015 CIS Annual Meeting: Immune Deficiency & Dysregulation North American Conference
3597: RESISTANT CUTANEOUS VASCULITIS IN HLA CLASS I DEFICIENCY
Daman Langguth, MBChB, FRACP, FRCPA Immunology, Sullivan Nicolaides Pathology, Brisbane, Australia
A 23 year old patient with known HLA Class I deficiency due to a novel missense mutation defect in the TAP2 gene resulting a in a premature stop codon had persistent and recurrent cutaneous vasculitis.
The treatment was unresponsive to standard approaches with Bactrim, dapsone, colchicine, Interferon alpha, and initially responded to intravenous immunogloubulin. A severe disease flare meant oral steroids and methotrexate were initiated though failed to control disease. Cyclosporin was started and there was an immediate response. Two years later presumed CNI toxicity lead to a decline in renal function. The dose of cyclosporin was reduced and methotrexate 10 mg added as adjunctive therapy. A change to a tacrolimus is being considered.