Abstract

We present a case of cutaneous involvement of monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), a malignancy that typically presents in the small intestine. A 74-year-old man presented with purpuric patches that began in his axillae and spread to his groin. Biopsies demonstrated a dermal-based infiltrate of large and atypical CD8+/ CD56+/GM3-/EBER- lymphocytes with minimal epidermotropism. At the time of diagnosis, the patient underwent a staging PET/CT scan, which demonstrated no evidence of systemic lymphoma. He subsequently underwent skin-directed therapy of skin lesions with resolution. Approximately one year later, he developed purpuric patches and ulcerations in the oral mucosa. An oral biopsy demonstrated findings similar to those in the skin, suggesting oral involvement. Repeat staging with PET/CT and a bone marrow biopsy was negative for lymphoma. Months later, he presented with small bowel perforation, and a jejunal biopsy at that time demonstrated a CD8+/CD56+/GM3-/EBER- lymphoma, most consistent with MEITL. In retrospect, his initial cutaneous and oral lesions likely represented a mucocutaneous manifestation of MEITL, which was not apparent on imaging, and endoscopy is the preferred diagnostic modality. Reports of cutaneous manifestations of MEITL are scarce, and we did not find any reports of MEITL presenting as purpuric lesions. This case illustrates that MEITL should be on the differential diagnosis of a skin biopsy demonstrating a non-epidermotropic CD8+/CD56+/GM3-/EBER- T-cell lymphoma, and that such a biopsy should also prompt consideration for endoscopy to evaluate for gastrointestinal involvement, since this condition often is not apparent in PET/CT scans.