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Pagetoid Reticulosis with Double-Negative CD4/CD8: A Rare Case Report
Hadeel Alsulimani1, Hamza Fadel2, Amal Alomari3
1King Fahad General Hospital, Jeddah, Saudi Arabia.
Introduction:
Pagetoid reticulosis (PR) is a rare variant of mycosis fungoides, characterized by localized patches or plaques mainly on the distal extremities with intraepidermal proliferation of neoplastic T cells. Typically, PR exhibits a CD4-positive, CD8-negative immunophenotype, whereas a CD4-/CD8- double-negative profile is distinctly uncommon, yet in localized pagetoid reticulosis it may be associated with an indolent clinical course and favorable therapeutic response.
Case Presentation:
In this report, we present a case of a healthy 58-year-old male with a 3-year history of a persistent erythematous plaque on the left forearm. Histopathological analysis demonstrated atypical lymphocytes with prominent epidermotropism. Immunohistochemical staining showed double-negative CD4/CD8 phenotype with positive CD3, CD5, and CD7. The lesion responded completely to topical therapy followed by localized radiotherapy, with clinical remission at follow-up.
Conclusion:
The CD4-/CD8- immunophenotype in localized pagetoid reticulosis is rare but may still be associated with an indolent clinical course and favorable therapeutic response.
