Article
Inherited Immunodeficiency: A New Association With Early-Onset Childhood Panniculitis.
Pediatrics - 1 Apr 2018
Bader-Meunier Brigitte, Rieux-Laucat Frédéric, Touzot Fabien, Frémond Marie-Louise, André-Schmutz Isabelle, Fraitag Sylvie, Bodemer Christine
Abstract excerpt
We report on 4 children who presented with aseptic panniculitis associated with inherited immunodeficiency. Three patients had a B-cell immunodeficiency resulting from mutations in the TRNT1 and NF-κb2 genes (no mutation was found in the third patient), and 1 had a T-cell deficiency (mutation in the LCK gene). Panniculitis occurred before the age of 2 years in the 4 patients and preceded the onset of recurrent...
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