Childhood primary cutaneous lymphoma: Case report of a rare dermatologic malignancy

Primary cutaneous T-cell lymphomas (CTCLs) are rare extranodal non-Hodgkin lymphomas, especially in children. Diagnosing CTCL in pediatric patients is challenging due to its clinical resemblance to inflammatory dermatoses and infections. We report a 15-year-old girl who presented with multiple erythematous to violaceous papulonodular, non-pruritic lesions over the face, abdomen, and extremities for 6 months, preceded by low-grade fever. The initial clinical impression was sarcoidosis. Hematological examination revealed microcytic hypochromic anemia. Fine needle aspiration cytology of the cutaneous lesion showed atypical lymphoid proliferation. Histopathology revealed a non-epidermotropic, folliculocentric, deep dermal infiltrate of atypical lymphoid cells. Immunohistochemistry showed CD45+, CD3+, CD5+, CD4−, CD8−, CD20−, CD30−, confirming primary CTCL of double-negative (CD4/CD8) phenotype. Primary CTCL in childhood is extremely rare and often misdiagnosed. High clinical suspicion and integration of histopathology with immunophenotyping are essential. Reporting such cases contributes to understanding their varied presentation and aids early diagnosis.