Inborn Error of Immunity-Associated Cutaneous T-Cell Lymphoproliferative Disorder

**Semantic type:** Neoplastic Process

**Definition:** A cutaneous EBV-negative lymphoproliferative disorder that is composed of CD8-positive T-cells and is associated with inborn immunodeficiency. The cutaneous manifestations include generalized papulo-nodular indurated lesions. It infrequently affects the subcutaneous tissue, lungs, bone marrow, and gastrointestinal tract as well. It has a variable clinical course.

**Synonyms:** - CD8-Positive Granulomatous Cutaneous T-Cell Lymphoma - Granulomatous CD8-Positive T-Cell Lymphoproliferative Disorder - IEI-Associated T LPD - Inborn Error of Immunity-Associated T Lymphoproliferative Disorder - Inborn Error of Immunity-Associated T-Cell Lymphoproliferative Disorder