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Cutaneous involvement from primary orbital lymphoma is uncommon. We report a patient with follicular lymphoma of the orbit who presented initially with cutaneous lesions clinically resembling lymphocytoma cutis which subsequently proved to be metastasis from the orbit.
A 75 year old woman presented to the dermatologist with a 6–7 month history of lumps on the right and left ears. Examination revealed two soft erythematous nodules, 2–3 cm in diameter behind the right ear and similar symmetrical lesions in both conchal bowls (Fig 1⇓). There was no lymphadenopathy or hepatosplenomegaly. Full clinical examination, chest x ray, full blood counts, erythrocyte sedimentation rate, U&E, and liver function tests were normal. A biopsy was performed. The clinical impression was that of lymphocytoma cutis and the lesions were treated with intralesional steroids resulting in prompt resolution leaving behind only flat discoloured areas. However, histology showed a nodular dermal lymphoid infiltrate of centrocyte-like cells with only scanty scattered blasts. Immunophenotypically, the lesional cells were of B cell phenotype (CD20+, CD79a+, CD10+, bcl6+, CD5−, CD43−, CD23−), overall in keeping with follicular lymphoma, grade 1. On staging investigations there was no evidence of lymphoma in other sites and the lesions were deemed to represent primary cutaneous disease.
Three months later the patient developed progressively enlarging conjunctival lesions near the medial canthus in both eyes (Fig 2A⇓). On examination she had bilateral subconjunctival fleshy lesions measuring about 5 mm. A computed tomograph (CT) scan showed a mass in the inferomedial aspects of the right orbit, extending down the nasolacrimal duct to the nasal cavity and a soft tissue mass confined to left orbit (Fig 2B⇓). The lesion was biopsied and histologically showed features identical to those in the biopsy of the skin lesion: sheets of centrocytes with scanty centroblasts. On immunoassay both the cutaneous and the orbital tumours showed the same immunophenotype (CD20+, CD10+, bcl6+, bcl2+, MT2+, CD5−, CD23, CD43−) in keeping with grade 1 follicular lymphoma. Polymerase chain reaction (PCR) analysis with primers for immunoglobulin heavy chain rearrangement revealed a prominent monoclonal band in an oligoclonal background. The initial biopsy of the cutaneous lesion on PCR analysis showed a monoclonal band of the same molecular weight. Staging confirmed this to be localised to the orbit but later another plaque-like cutaneous lesion (1 cm in diameter) appeared on her forehead with the same histological, immunophenotypic, and molecular genetic features. The patient was commenced on chlorambucil chemotherapy with prompt resolution of the orbital and cutaneous lesions. She remains well at 18 months of follow up.
In this unusual case of orbital lymphoma the initial presentation was in the skin. On clinical examination the cutaneous changes were deemed to represent lymphocytoma cutis. The term lymphocytoma cutis stands for a highly heterogeneous group of reactive lymphoid proliferations in the skin. These include Borrelia burgdorferi associated lesions,1 post-zoster scar reactions,2 trauma,3,4 and those of unknown aetiology.3,4 Clinically, they are characterised by flesh coloured to plum red dermal and subcutaneous nodules and plaques as in our case. They can be solitary or multiple. It is more common females (F:M = 3:1),3 mostly involving the face (70%). Lymphocytoma cutis has also been reported with conjunctival lesions.5 We would like to stress that despite typical clinical appearance before diagnosis of lymphocytoma cutis is made, full pathological investigation with immunophenotyping and molecular genetic clonality analysis is essential.
In the interval before the clinical appearance of the orbital tumours in our patient the cutaneous lesions were considered to represent a primary follicular lymphoma at this site. This accounts for 10% of cutaneous B cell neoplasms and follows a very indolent clinical course during which it remains confined to the skin.6 Distinction between primary and secondary involvement is of paramount importance. Secondary cutaneous involvement by follicular lymphoma is not associated with such a favourable prognosis. Morphologically there is no difference between the two; however, the primary cutaneous type is usually bcl-2 negative on immunostaining and the t (14; 18) is hardly ever found. It is therefore regarded by some authors as part of the spectrum of cutaneous marginal zone lymphomas6 rather than follicular lymphoma.
At clinical presentation of the orbital tumours in our patient, as recommended by EORTC 7 the lesions in the skin were deemed to represent secondary involvement from the primary site in the orbit though orbital lesions were recognised later. On PCR analysis for B cell clonality it was apparent that the two lesions represented the same tumour. Follicular lymphoma in the orbit accounts for 20%–33%8 of all lymphomas in this site. Its pathological features in the orbit are no different from the lymph node counterparts and, unlike those in the skin, are bcl-2 positive and bare the t (14; 18). Early extraorbital spread is regarded a sign of more aggressive behaviour and a potential indicator for poorer prognosis. Secondary cutaneous involvement from a primary orbital lymphoma is an uncommon event with only one case described in the literature9 while the primary cutaneous lymphomas occurs more frequently.10