Abstract

Lymphomatoid papulosis (LyP) is a chronic recurrent self-healing skin disease characterized by waxing and waning papules and nodules and is one of the CD30-positive T-cell lymphoproliferative disorders. Historically, three subtypes were recognized and well-studied (A, B and C). After 2010, the rare types D, E and F were described. Herein, we report a 64-year-old male who presented with waxing and waning papules and plaques over a period of 3 weeks. The lesions tend to heal then popped up in new places. His past medical history was remarkable for multiple myeloma status post stem cell transplantation. Skin biopsy was performed to rule out graft vs host disease and drug eruption. Sections revealed a predominantly epidermotropic, atypical small to medium-sized CD3-positive T-cell infiltrate that was positive for CD8 and CD30, and negative for CD4. T-cell receptor (TCR) gene rearrangement studies were positive for clonal T-cell population. The cytotoxic markers, TIA1 and Granzyme B were positive. The cells were immunopositive for TCR beta F1, and negative for TCR delta. The cells were focally positive for MUM1 and BCL2 immunostaining. The histologic differential diagnosis included primary cutaneous aggressive epidermotropic CD8+ cytotoxic T-cell lymphoma, which portends dismal prognosis (median survival 32 months) and CD8 positive mycosis fungoides. The distinction is very important for prognostication as well for management of these patients. The clinicopathologic correlation is critical for establishing an accurate diagnosis of LyP type D, as these patients only need to be observed and followed up.