Improvement of toxic epidermal necrolysis after the early administration of a single high dose of intravenous immunoglobulin.

BACKGROUND

Toxic epidermal necrolysis (TEN) is a severe disease often induced by drugs. Treatment is controversial, although intravenous immunoglobulins (IVIGs) have been effective.

OBJECTIVE

To report the case of a child with TEN after lamotrigine treatment, who improved 24 hours after IVIG administration.

METHODS

Sequential blood and blister fluid samples were obtained for flow cytometry and reverse transcriptase-polymerase chain reaction analyses.

RESULTS

The first blood sample, taken before IVIG administration, showed normal levels of lymphocyte subsets and CLA (4.0%) but high levels of activated lymphocytes (CD69) (18.0%). After treatment, the CLA+, CD69+, and memory cells increased until day 7, decreasing to normal values at days 15 and 30. In the blister fluid samples, taken on day 1, there were high levels of CD8+ (70.2%; CD4/CD8 ratio, 1:5), CLA+ (18.8%), and CD69+ (70%) cells, decreasing 24 hours after IVIG administration. In the blood samples, there was a Th1 cytokine pattern initially, tending to Th0 with time. Perforin, granzyme B, and Fas ligand were only observed before IVIG administration.

CONCLUSIONS

A single high dose of IVIG interrupted the progression of skin disease and reduced the expression of the apoptotic markers. The immunologic changes, first seen in blister fluid and remaining several days in peripheral blood, indicate that T cells were first recruited to the skin and then recirculated to blood.