Cutaneous T-cell lymphoma in the setting of NIPAL4-related nonsyndromic epidermal differentiation disorder: three cases

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ID: 321870
2026
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Abstract
We report three patients with nonsyndromic epidermal differentiation disorders associated with the same recurrent pathogenic NIPAL4 variantt who subsequently developed cutaneous T-cell lymphoma (Sézary syndrome in all cases). These observations suggest a possible link between congenital epidermal barrier defects and lymphomagenesis, and highlight the importance of considering skin biopsy in nEDD patients with phenotypic change, pruritus, or treatment resistance.
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openalex_W7170079834 Use this key to autocite in the manuscript while using SciMatic Manuscript Manager or Thesis Manager
Authors Quitterie Murat de Montai, Emmanuelle Bourrat, Gilles Battesti, G. Hickman, Pauline Bataille, S. Miskinyte, Asma Smahi, Hélène Moins-Teisserenc, C Grolleau, Smail Hadj-Rabia, C Ram-Wolff, Bouaziz Jd, Christine Bodemer, Judith Fischer, Alain Hovnanian, Maxime Battistella, A de Masson, Alizée Bozonnat
Journal the british journal of dermatology
Year 2026
DOI
10.1093/bjd/ljag302
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