TY - JOUR
T1 - Intertriginous mycosis fungoides
T2 - A distinct presentation of cutaneous T-cell lymphoma that may be caused by malignant follicular helper T cells
AU - Gammon, Bryan
AU - Guitart, Joan
N1 - Copyright:
Copyright 2013 Elsevier B.V., All rights reserved.
PY - 2012/9
Y1 - 2012/9
N2 - Background: Follicular helper T cells are a subset of helper T cells that facilitate B-cell recruitment and maturation. Rare cases of cutaneous T-cell lymphoma manifesting as de novo tumor lesions in intertriginous skin contain an infiltrate rich in B cells. These cases may represent malignant counterparts of skin-homing follicular helper T cells. Observations: Two men and 1 woman (age range, 35-58 years) were seen with predominantly intertriginous tumor-stage cutaneous T-cell lymphoma lesions characterized by the absence of epidermotropism and the presence of a mixed infiltrate rich in B cells. Two of the patients died of the disease less than 3 years from the initial diagnosis. The surviving patient has aggressive disease and underwent hematopoietic stem cell transplantation. Two of the patients had a prominent CXCL13+, Bcl6/CD3+, and programmed death protein 1-positive follicular helper T-cell population. Conclusions: The intertriginous tumor variant of cutaneous T-cell lymphoma is heterogeneous but may be associated in some cases with a follicular helper T-cell immunophenotype. These patients may follow an aggressive clinical course. Tumor progression in sanctuary sites on patients receiving phototherapy may manifest as a similar clinical phenotype. Further characterization of the disease process is needed to confirm this observation.
AB - Background: Follicular helper T cells are a subset of helper T cells that facilitate B-cell recruitment and maturation. Rare cases of cutaneous T-cell lymphoma manifesting as de novo tumor lesions in intertriginous skin contain an infiltrate rich in B cells. These cases may represent malignant counterparts of skin-homing follicular helper T cells. Observations: Two men and 1 woman (age range, 35-58 years) were seen with predominantly intertriginous tumor-stage cutaneous T-cell lymphoma lesions characterized by the absence of epidermotropism and the presence of a mixed infiltrate rich in B cells. Two of the patients died of the disease less than 3 years from the initial diagnosis. The surviving patient has aggressive disease and underwent hematopoietic stem cell transplantation. Two of the patients had a prominent CXCL13+, Bcl6/CD3+, and programmed death protein 1-positive follicular helper T-cell population. Conclusions: The intertriginous tumor variant of cutaneous T-cell lymphoma is heterogeneous but may be associated in some cases with a follicular helper T-cell immunophenotype. These patients may follow an aggressive clinical course. Tumor progression in sanctuary sites on patients receiving phototherapy may manifest as a similar clinical phenotype. Further characterization of the disease process is needed to confirm this observation.
UR - http://www.scopus.com/inward/record.url?scp=84866511730&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84866511730&partnerID=8YFLogxK
U2 - 10.1001/archdermatol.2012.1545
DO - 10.1001/archdermatol.2012.1545
M3 - Article
C2 - 22986857
AN - SCOPUS:84866511730
SN - 2168-6068
VL - 148
SP - 1040
EP - 1044
JO - A. M. A. archives of dermatology and syphilology
JF - A. M. A. archives of dermatology and syphilology
IS - 9
ER -