Conclusion and Results (Outcome and follow-up)
Due to recurrent skin and systemic infections in infancy and associated erythroderma, the child was started on intravenous immunoglobulin, as subcutaneous administration was judged not possible in this child, to reduce infectious exacerbations, improve local skin status, and improve quality of life. Our patient, like most NS patients, has normal serum IgG levels, suggesting that the improvement with replacement therapy in these patients is more likely due to the immunomodulatory effects of IVIG. Recent data show that the number of pediatric patients with NS treated with immunoglobulins was 14, of whom 12 showed a positive clinical effect, with the dose administered ranging from 400 to 500 mg/kg per month and the duration of treatment from 2 months to 5 years.9 A recent meta-analysis comparing different IVIG infusion regimens in patients with Kawasaki disease found that high-dose IVIG regimens are likely to be more effective and may even reduce the need for additional treatment compared to medium- or low-dose IVIG regimens, with little or no difference in the number of adverse events between groups.10 We decided to test whether the higher IVIG dose would not be more effective in patients with NS. We started with 250 mg/kg/day for 3 days or 750 mg/kg/month administration and have not observed any adverse effects so far.
During the follow-up period, normalization of liver transaminases was noted, and another interesting phenomenon was the sustained decrease in total IgE (> 2500 – > 484. 5 – > 256 – > 183. 3 – > 160. 2 – > 129.2 IU/ml). It may represent a marker of favorable therapeutic response. No such positive trend was observed with vitamin D supplementation, and the child maintained reduced levels (51 nmol/l) despite oral supplementation. In the last five months since receiving replacement therapy, the child gained two kilograms and grew three centimeters (BMI 18 P, -0.91 z). Furthermore, decrease in the incidence of infectious exacerbations was noted in addition to significant reduction of the Netherton Area and Severity Assessment (NASA) score (66 to 36 points) (Figure 4).