An aggressive primary orbital natural killer/T-cell lymphoma case: poor response to chemotherapy.

Natural killer/T-cell lymphoma (NKTCL) and its presentation with extranodal orbital involvement as a single lesion are extremely rare. The aim of this article was to describe the presentation, diagnosis, and systemic treatment of a primary orbital NKTCL. A 67-year-old Caucasian woman presented with left exophthalmos, pain, periorbital swelling, and limited extrinsic ocular motility. Orbital cellulitis was suspected, but finally orbital biopsy was performed due to no response to initial antibiotic and anti-inflammatory standard treatment. The pathologic diagnosis was NKTCL. Systemic evaluations were negative. CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) chemotherapy was initiated, but after 2 cycles of treatment, tumoral progression was observed. SMILE (dexamethasone, methotrexate, ifosfamide, L-asparaginase, etoposide) rescue chemotherapy was then administered. Lymphoma progression was inevitable. She died 10 months later. Although more nasal NKTCL cases have been described, the nonnasal primary orbital NKTCL is an uncommon neoplasm with high mortality rate, despite the recent use of more potent chemotherapy regimens.

Eyelid lesions in lipoid proteinosis or Urbach-Wiethe disease: case report and review of the literature.

Lipoid proteinosis (LP) or Urbach-Wiethe disease is a recessively inherited disorder not usually seen by ophthalmologists. It is characterized by non-inflammatory, persistent papules on the skin and mucous membranes. The first clinical manifestation of LP is usually progressive hoarseness. The lid lesions, consisting of beaded papules along the lid margins (moniliform blepharosis), are considered almost pathognomonic of the disease. The interesting clinical and histopathological features of LP are described in a 45-year-old man with a history of lipoid proteinosis, who presented to us for evaluation of ocular discomfort and unusual multiple eyelid lesions. We surgically removed all the eyelid lesions. The histologic findings were consistent with LP. Knowledge of the typical eyelid lesions may help to diagnose many typical or atypical cases of LP, although biopsy confirmation is always necessary. In our experience, the surgical removal of the eyelid lesions seems to be curative and due to the amount of eyelid lesions that can be developed, it is recommended to initiate an early treatment as soon as possible to avoid postoperative deformities.