Cutaneous Manifestation of Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma: A Clue to A Gastrointestinal Malignancy.

We present a rare case of cutaneous involvement of monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL). A 68-year-old man first presented with multiple purpuric patches which extended from his axilla to his groin. Biopsies demonstrated a dermal-based infiltrate of predominantly medium-sized atypical CD8+/CD56+/TCR-δ-/EBER- T-lymphocytes with slight epidermotropism associated with extravasated red cells. A diagnosis of primary cutaneous cytotoxic T-cell lymphoma was rendered after presumably excluding systemic lymphoma by staging PET/CT scan and bone marrow biopsy. One year later, he developed purpuric patches and ulcerations of the tongue and 3 months after that, jejunal perforation. Both tongue biopsy and small bowel resection revealed a similar infiltrate of epitheliotropic CD8+/CD56+/TCR-δ-/EBER- cytotoxic T-cells. Supportive histopathologic features and the absence of gluten-sensitive enteropathy eventually led to the diagnosis of MEITL in the jejunal resection. In retrospect, his initial cutaneous and oral lesions were a mucocutaneous manifestation of MEITL as well, and systemic involvement was not detected on conventional staging. Cutaneous involvement in MEITL is exceedingly uncommon, and this case underscores the importance of including MEITL in the differential diagnosis of skin biopsies of mostly dermal-based lymphomas with only slight epidermotropism with a CD8+/CD56+/TCRδ-/EBER- phenotype. Early endoscopic evaluation may facilitate diagnosis of occult intestinal disease.