Table 1.
Age | Disease status | Therapy |
---|---|---|
15 months | NOD2 mutation discovered. Severe papular dermatitis, pruritus, arthritis, uveitis |
Started on oral prednisolone 2 mg/kg/day for 8 weeks |
17 months | Resolved dermatitis, pruritus, arthritis, and uveitis | Finished first course of oral steroids |
19 months | Recurrence of dermatitis, pruritus, arthritis, and uveitis | Re-initiation of second course of oral prednisolone 2 mg/kg/day for 8 weeks |
21 months | Resolved dermatitis, pruritus, arthritis, and uveitis | Finished second course of corticosteroids |
22 months | Recurrence of dermatitis, pruritus, arthritis, and uveitis | Weaned off steroids Started on 7.5 mg methotrexate subcutaneously weekly + 1 mg folic acid orally daily |
24 months | Improved dermatitis, but persistent arthritis, pruritus, and uveitis | Started 75 mg daily hydroxychloroquine |
26 months | Resolved dermatitis, pruritus, arthritis, and uveitis | Hydroxychloroquine discontinued due to desire to minimize long-term macular toxicity/risk for retinopathy Continued on methotrexate 7.5 mg subcutaneously weekly |
30 months | Remains free of dermatitis, pruritus, arthritis, and uveitis | Continuing 7.5 mg methotrexate subcutaneously weekly + 1 mg folic acid orally daily |