Abstract

A 55-year-old female without a significant past medical history was referred to the dermatology clinic for a lesion on the face of about four months duration. She denied associated symptoms. On physical examination, there was a 9 mm x 6 mm ovoid, pink to violaceous, smooth dome-shaped papule on the left nasal side wall. A 4-mm punch biopsy to the center of the papule was obtained. Hematoxylin and eosin stained sections revealed a dense dermal lymphoid infiltrate extending to the deep dermis. Focal epidermotropism was present. The infiltrate consisted of small to intermediate sized lymphocytes and scattered large hyperchromatic forms with ovoid nuclei and small nucleoli. Background histiocytes and few plasma cells were presenting. Immunohistochemistry revealed a predominance small and large T-cells without loss of pan T-cell markers CD2, CD3, CD5 or CD7. The cells were mostly CD4 positive with a few CD8 positive cells. CD20 showed background small B-cells. Kappa and Lambda demonstrated scattered polytypic plasma cells. No germinal centers were highlighted with CD10 or BCL6. No aberrant expression of Cyclin D1, BCL2, or CD30 were identified. Immunoglobulin heavy-chain and T-cell receptor gene rearrangement studies were negative. Given the clinical and histologic findings the diagnosis was most consistent with a primary cutaneous CD4+ small/medium sized pleomorphic T-cell lymphoma was made. This is a rare cutaneous neoplasm with few cases reported. It is thought to have a favorable prognosis without systemic involvement. Among other T-cell rich proliferations, it is important to be able to distinguish this entity from tumor-stage MF, lymphomatoid papulosis, and subcutaneous panniculitis-like T-cell lymphoma. We present a rare case of primary cutaneous CD4+ small/medium sized pleomorphic T-cell lymphoma and discuss its clinical course and diagnostic work-up. Clinical and histological images, along with clinical follow-up and treatment will be presented.

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