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History
A 63-year-old man presents to the dermatology out-patient clinic with a slowly growing nodule on his back. He had been unaware of it but his wife is worried as she feels that it has grown over the past year. There is no previous history of skin problems. He takes amlodipine, aspirin and simvastatin and is systemically well.
Examination
There a 3 cm 3 cm red to plum coloured, firm, non-tender, solitary nodule with a smooth surface on his back (Fig. 66.1). Full skin examination does not reveal any further lesions and there is no evidence of peripheral lymphadenopathy.
Questions
• What are the clinical differential diagnoses?
• How should the patient be managed?
• What is the prognosis?
Medicine
The differential diagnoses of a firm, indurated erythematous purple papule/nodule that remains fixed for a period of time on the trunk include sarcoid, cutaneous metastasis B-cell lymphoma, Kaposi’s sarcoma, and a keloid scar. Skin biopsy with histopathology and immunophenotyping should help to clarify the diagnosis. This patient was diagnosed with cutaneous B-cell lymphoma, CBCL (a primary cutaneous follicle centre cell lymphoma). CBCL develops when a clonal proliferation of B lym-phocytes is confined to the skin. Primary cutaneous lymphomas of the B-cell type comprise approximately 20 per cent of cutaneous lymphomas. Other types of CBCL include primary cutaneous marginal zone, primary cutaneous large B-cell, leg type, and primary cutaneous diffuse large B-cell lymphomas. Follicle centre cell lymphomas are clinically characterized as asymptomatic nodules, plaques or tumours. CBCL lesions are slow-growing, usually red to plum in colour and are firm with a smooth surface. Common sites include the trunk, scalp and forehead. Primary CBCL needs to be differentiated from a secondary cutaneous lymphoma, which is where a nodal follicular lymphoma has metastasized to involve the skin. This can be differentiated by molecular techniques to demonstrate a lack of the t (14;18) chromo-somal translocation.
For solitary lesions or small groups, the treatment of choice is local radiotherapy. For those patients with multiple lesions at distant sites, then chemotherapy may be required. The prognosis is excellent with a 5-year survival of more than 95 per cent. For the other cutaneous subtypes, patients should be investigated thoroughly for nodal and extracutaneous disease. These investigations include peripheral blood studies, bone marrow biopsy, radiographic imaging and, if indicated, lymph node biopsy.
KEY POINTS
• Cutaneous B-cell lymphoma (CBCL) occurs when a clonal proliferation of B lymphocytes is confined to the skin.
• Follicle centre cell lymphomas are characterized as asymptomatic nodules.
• Prognosis is excellent with a 5-year survival of more than 95 per cent.
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