A Gastrointestinal Manifestation of Primary Cutaneous Aggressive Epidermotropic Cytotoxic T-Cell Lymphoma: A Case...

Cutaneous T-cell lymphomas (CTCL) are the most frequently encountered form of primary cutaneous lymphoma (PCL). Although this group of diseases primarily affects the skin, extracutaneous manifestations are reported. Primary cutaneous aggressive epidermotropic cytotoxic T-cell lymphoma (pcAECTCL) is a rare form of CTCL that usually presents with an aggressive course and poor prognosis. Given the scarcity of cases, the disease is poorly understood. There have been reports of cases of extracutaneous manifestations of the disease, with more aggressive courses associated with an increased propensity for systemic spread. The most common extracutaneous manifestations of pcAECTCL include those affecting the lungs, central nervous system, testis, and oral mucosa, which can cause progressive dysphagia. We describe a patient with a chronic relapsing/remitting rash diagnosed as pcAECTCL. A positron emission tomography (PET) scan revealed diffuse uptake within the gastric region. Initial esophagogastroduodenoscopy (EGD) showed multiple ulcerations and erosions in the stomach with concern for extracutaneous spread of the patient's pcAECTCL. However, no definitive histopathological or flow immunophenotypic evidence of metastasis was found on the initial or subsequent repeat EGD.