Paraneoplastic pemphigus associated with post-transplant lymphoproliferative disorder after small bowel transplantation

Sander A. R. Fidder*, Marieke C. Bolling, Gilles F. H. Diercks, Hendri H. Pas, Louise H. L. Hooimeijer, Laura B. Bungener, Brigitte W. M. Willemse, Rene Scheenstra, Janneke M. Stapelbroek, Hubert P. J. van der Doef

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

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Background PNP is a malignancy-associated autoimmune mucocutaneous syndrome due to autoantibodies against plakins, desmogleins, and other components of the epidermis and basement membrane of epithelial tissues. PNP-causing malignancies comprise mainly lymphoproliferative and hematologic neoplasms. PNP is extremely rare, especially in children.

Methods Here, we present the first case of a child who developed PNP on a PTLD after small bowel transplantation because of a severe genetic protein-losing enteropathy.

Results The patient in this case report had a severe stomatitis, striate palmoplantar keratoderma, and lichenoid skin lesions. In addition, she had marked esophageal involvement. She had lung pathology due to recurrent pulmonary infections and ventilator injury. Although we found no evidence of BO, she died from severe pneumonia and respiratory failure at the age of 12 years.

Conclusion It is exceptional that, despite effective treatment of the PTLD, the girl survived 5 years after her diagnosis of PNP. We hypothesize that the girl survived relatively long after the PNP diagnosis due to strong T-cell suppressive treatments for her small bowel transplantation.

Original languageEnglish
Article number14023
Pages (from-to)e14023
Number of pages6
JournalPediatric transplantation
Issue number6
Publication statusPublished - Sept-2021


  • antibodies
  • children
  • intestine transplant
  • liver
  • malignancy
  • organ transplantation
  • paraneoplastic pemphigus
  • PTLD
  • SKIN

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