Cutaneous γδ T-Cell Lymphoma With Bilateral Ocular and Adnexal Involvement

Subcutaneous panniculitis-like γδ T-cell lymphoma (SPTL-GD) is a rare subtype of primary cutaneous non-Hodgkin T-cell lymphoma caused by a clonal proliferation of mature activated cytotoxic γδ T cells.^1,2 It typically manifests with skin nodules or plaques over the extremities and has an aggressive clinical course.² Although rare, reports exist of other metastatic cutaneous T-cell lymphomas with periocular or intraocular manifestations.^3,4However, to our knowledge, we report the first case of SPTL-GD manifesting this way. Furthermore, the simultaneous adnexal, intraocular, and neuro-ophthalmic involvement described here has not been reported for any manifestation of ocular lymphoma.

A 62-year-old woman had a 7-month history of bilateral red eyes with blurred vision. She was diagnosed as having bilateral nongranulomatous anterior uveitis and began treatment with topical steroid eyedrops. However, her vision continued to slowly deteriorate and she additionally began to experience night sweats, fevers, and weight loss. Two months later, she also noticed 2 firm swellings by her right eyebrow and described sequential bilateral pupillary enlargement.

When examined at this stage, her visual acuity was counting fingers OD and 20/80 OS. Both pupils measured 4.5 mm and were poorly reactive to light with no light-near dissociation but hypersensitivity to pilocarpine, 0.125%. There was no ptosis, eyelid malposition, proptosis, globe displacement, or limitation of eye movements. Anterior segment examination revealed bilateral punctate epitheliopathy with reduced corneal sensation, corneal edema, and no infiltrates. There were multiple small nongranulomatous keratic precipitates with 2+ to 3+ of anterior chamber cells in both eyes but no fibrin or hypopyon. Views of the posterior segments were difficult but showed no abnormalities.

Facial examination revealed 2 right subbrow, subcutaneous lesions that were well circumscribed, firm, nontender, and immobile (Figure 1A). No lymph nodes were palpable, and no other systemic abnormalities were detected. However, during the ensuing weeks, 2 other smaller subcutaneous masses developed around the right lower punctum and left cheek.

Her erythrocyte sedimentation rate, C-reactive protein level, full blood cell count, and renal and liver function test results were normal except for an increased platelet count of 679 × 10³/μL (reference range, 150-400 × 10³/μL; to convert to ×10⁹/L, multiply by 1.0) and a serum angiotensin-converting enzyme level of 124 U/L (reference range, 8-65 U/L; to convert to nanokatals per liter, multiply by 16.667). Computed tomographic scans of the head, chest, abdomen, and pelvis were normal, but magnetic resonance imaging of the head revealed smooth perineural enhancement of both optic nerve sheaths along their intraorbital portion (Figure 1B). An anterior chamber paracentesis was performed and fluid was negative for herpes simplex virus, varicella-zoster virus, cytomegalovirus, and Epstein-Barr virus DNA on polymerase chain reaction. Insufficient sample was available for cytology.

Excisional biopsies of the right subbrow lesions were performed; they were difficult to interpret because of severe necrosis. However, subsequent specimens from the left cheek and right lower eyelid lesions confirmed SPTL-GD (Figure 2). Lesional T cells expressed CD3, CD7, CD30, perforin, TIA-1, and granzyme B and were negative for CD4, CD8, CD56, T-cell receptor α/β (BF-1), and Epstein-Barr virus–encoded small RNA on immunophenotyping/in situ hybridization. The proliferation index as assessed by MIB-1 was 90%.

Ophthalmic manifestations of lymphoma are broad and include both ocular adnexal and intraocular disease. Most are B-cell non-Hodgkin lymphomas, although the intraocular and adnexal manifestations relate to different subtypes and do not coexist.⁵ T-cell lymphomas, including primary cutaneous ones, have also been reported to involve the eye. Typically, they have eyelid involvement and occasionally have intraocular manifestations such as nonspecific uveitis or retinal infiltrates.³ Our case is unusual owing to the simultaneous manifestation of ocular adnexal, intraocular, and neuro-ophthalmic pathologic findings. Although the only biopsy-proven site of involvement in our case was the ocular adnexae, both papillitis and infiltrative optic neuropathy have separately been reported with cutaneous T-cell lymphoma, as have anterior uveitis, corneal involvement, and pupillary dilation.^3,4 Finally, SPTL-GD as a specific subtype of cutaneous T-cell lymphoma has previously been reported only with extraocular muscle involvement.⁶

Correspondence: Dr A. M. S. Morley, Department of Ophthalmology, St Thomas' Hospital, Westminster Bridge Road, London SE1 7EH, England (susie.morley@gstt.nhs.uk).

Additional Contributions: Ata Siddiqui, FRCR, helped with the radiological imaging.