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

doi:10.1111/petr.14023

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 journal › Article › Academic › peer-review

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Abstract

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 language	English
Article number	14023
Pages (from-to)	e14023
Number of pages	6
Journal	Pediatric transplantation
Volume	25
Issue number	6
DOIs	https://doi.org/10.1111/petr.14023
Publication status	Published - Sept-2021

Keywords

antibodies
children
intestine transplant
liver
malignancy
organ transplantation
paraneoplastic pemphigus
PTLD
BRONCHIOLITIS OBLITERANS
ANTIBODIES
DIAGNOSIS
AUTOANTIBODIES
NEOPLASMS
DISEASE
SKIN

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Fidder, S. A. R., Bolling, M. C., Diercks, G. F. H., Pas, H. H., Hooimeijer, L. H. L., Bungener, L. B., Willemse, B. W. M., Scheenstra, R., Stapelbroek, J. M., & van der Doef, H. P. J. (2021). Paraneoplastic pemphigus associated with post-transplant lymphoproliferative disorder after small bowel transplantation. Pediatric transplantation, 25(6), e14023. Article 14023. https://doi.org/10.1111/petr.14023

@article{530e384a794c4fcaaeb38ce5ce00a585,

title = "Paraneoplastic pemphigus associated with post-transplant lymphoproliferative disorder after small bowel transplantation",

abstract = "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.",

keywords = "antibodies, children, intestine transplant, liver, malignancy, organ transplantation, paraneoplastic pemphigus, PTLD, BRONCHIOLITIS OBLITERANS, ANTIBODIES, DIAGNOSIS, AUTOANTIBODIES, NEOPLASMS, DISEASE, SKIN",

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

note = "{\textcopyright} 2021 The Authors. Pediatric Transplantation published by Wiley Periodicals LLC.",

year = "2021",

month = sep,

doi = "10.1111/petr.14023",

language = "English",

volume = "25",

pages = "e14023",

journal = "Pediatric transplantation",

issn = "1397-3142",

publisher = "Blackwell Publishing Inc.",

number = "6",

}

Fidder, SAR, Bolling, MC , Diercks, GFH, Pas, HH, Hooimeijer, LHL, Bungener, LB , Willemse, BWM , Scheenstra, R, Stapelbroek, JM & van der Doef, HPJ 2021, 'Paraneoplastic pemphigus associated with post-transplant lymphoproliferative disorder after small bowel transplantation', Pediatric transplantation, vol. 25, no. 6, 14023, pp. e14023. https://doi.org/10.1111/petr.14023

TY - JOUR

T1 - Paraneoplastic pemphigus associated with post-transplant lymphoproliferative disorder after small bowel transplantation

AU - Fidder, Sander A. R.

AU - Bolling, Marieke C.

AU - Diercks, Gilles F. H.

AU - Pas, Hendri H.

AU - Hooimeijer, Louise H. L.

AU - Bungener, Laura B.

AU - Willemse, Brigitte W. M.

AU - Scheenstra, Rene

AU - Stapelbroek, Janneke M.

AU - van der Doef, Hubert P. J.

PY - 2021/9

Y1 - 2021/9

N2 - 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.

AB - 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.

KW - antibodies

KW - children

KW - intestine transplant

KW - liver

KW - malignancy

KW - organ transplantation

KW - paraneoplastic pemphigus

KW - PTLD

KW - BRONCHIOLITIS OBLITERANS

KW - ANTIBODIES

KW - DIAGNOSIS

KW - AUTOANTIBODIES

KW - NEOPLASMS

KW - DISEASE

KW - SKIN

U2 - 10.1111/petr.14023

DO - 10.1111/petr.14023

M3 - Article

C2 - 34014017

SN - 1397-3142

VL - 25

SP - e14023

JO - Pediatric transplantation

JF - Pediatric transplantation

IS - 6

M1 - 14023

ER -

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

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