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CD30+ anaplastic large cell lymphoma (ALCL) belongs to the group of T cell non-Hodgkin’s lymphomas. The primary cutaneous variant of ALCL usually presents as a solitary, cutaneous, or subcutaneous reddish violet lesion, which can be superficially ulcerated. We present the case of a solitary CD30+ ALCL of the eyelid showing regression.
A 39 year old man presented with a 4 week history of a progressive painless ulcerating nodule on the right upper eyelid, unresponsive to oral flucloxacillin. He was systemically well and denied recent foreign travel or contact with animals.
A 17 mm diameter ulcer with rolled margins and serosanguinous exudate was evident over the right upper eyelid (fig 1). His cornea, conjunctiva, and anterior chamber were normal. Systemic examination was unremarkable.
Investigations including full blood count, urea and electrolytes, bone and liver profile, immunoglobulins and electrophoresis, autoantibody screening, and Treponema antibody were normal or negative. Tissue culture failed to demonstrate a bacterial, viral, or fungal pathogen. There was no clinical, radiological, or bone marrow evidence of extracutaneous disease.
Histology of the biopsy taken from the lid ulcer margin showed epidermal necrosis associated with ulceration (fig 2). The ulcer base showed haemorrhagic granulation tissue infiltrated by a mixture of lymphocytes, plasma cells, neutrophils, and eosinophils. There were also ill defined groups of large blast cells showing enlarged and pleomorphic nuclei and high mitotic activity. The immunohistochemical staining showed these cells to be of T cell lymphoid lineage. Many of the large blast cells were CD30 positive but negative for ALK-1 protein. The features were of a CD30 positive anaplastic large cell lymphoma (ALK negative).
Treatment options such as surgery and radiotherapy were discussed with the patient but as the lesion remained stable over a 10 day period, a conservative approach was agreed. A moderately potent topical corticosteroid (mometasone furoate 0.1% cream) was applied to the lesion once daily. When followed up 8 weeks later the ulcer had completely healed without scarring (fig 3). Eighteen months has elapsed since presentation. There has been no recurrence of his disease, and he remains in good health.
ALCL represents a group of large cell lymphomas. They consist of a proliferation of predominantly large lymphoid cells with strong expression of the cytokine receptor CD30 (>75%). Using molecular and clinical criteria, three entities have been identified: primary systemic anaplastic lymphoma kinase (ALK) + ALCL, primary systemic ALK − ALCL, and primary cutaneous ALCL.1
Primary cutaneous ALCL arise de novo in the skin, commonly on the head and neck of older patients with a median age of 60 years and a male/female ratio of 3:2. Most patients present with solitary, asymptomatic nodules, which can be superficially ulcerated. Primary cutaneous ALCL has a more favourable prognosis than systemic ALCL, with an 5 year survival of approximately 90%. Partial or complete spontaneous regression can be observed in up to 25% of patients with primary cutaneous ALCL, accounting for the previous designation of “regressing atypical histiocytosis.” Treatment of localised lesions usually includes excision with or without radiation. However, patients with disseminated skin disease may benefit from systemic polychemotherapy.2,3 In our patient the lesion had resolved within 3 months of initial appearance. The application of a moderate potent topical steroid might have contributed to the regression of the ulcer.
We present a case of a primary cutaneous ALCL of the eyelid showing regression. Ophthalmologists should be aware of this sometimes self regressing entity and an expectant policy might be indicated in non-progressing tumours, thus avoiding potentially mutilating surgery or radiotherapy.
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