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).