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Intravascular large B‐cell lymphoma presenting as a nondescript macular eruption

Intravascular large B-cell lymphoma is a clinically aggressive subtype of extra-nodal, non-Hodgkin lymphoma. The classic variant presents with predominantly cutaneous findings and neurological deficits, whereas the haemophagocytic syndrome-associated variant presents with multiorgan failure, hepatosplenomegaly, pancytopenia and pyrexia of unknown origin. Cutaneous manifestations are reported in approximately 40% of all patients. We describe a patient with intravascular large B-cell lymphoma who presented with an asymptomatic telangiectatic macular eruption. A 65-year-old Malay male patient, without significant oncological history, first presented with a 1-week history of diarrhoea and 20 kg weight loss over 6 months. Physical examination revealed lower limb oedema, hepatosplenomegaly and ascites. No focal neurological deficits were elicited. An initial computed tomography scan of his thorax, abdominal and pelvis revealed findings suggestive of portal hypertension. Viral hepatitis serologies and full blood examination were unremarkable. He requested discharge but was readmitted 3 months later with a 1-week history of intermittent fevers. His tests showed raised creatinine (142 lmol/L), hypocalcaemia (adjusted calcium 2.05 mmol/L), hyperphosphatemia (2.07 mmol/L), raised lactate dehydrogenase (>2500 U/L) and raised uric acid (467 umol/L), suggestive of tumour lysis syndrome. Positron emission tomography, bone marrow biopsy and various microbiology investigations (including repeated blood cultures, cryptococcal antigen, CMV IgM, parvovirus IgM, bartonella serology, brucella antibody, histoplasma, EBV, sputum AFB smears and sputum TB PCR) were unyielding. He was referred to Dermatology for an asymptomatic rash over his chest of 2-month duration. Examination revealed telangiectatic and erythematous, blanchable, flat macules coalescing across his d ecolletage (Fig. 1). Two lesional biopsies over the chest revealed atypical large cells with large nuclei and mitotic figures within the dermal blood vessels (Fig. 2a). The cells stained positive for CD20 (Fig. 2b) and MUM-1 (Fig. 2c) and negative for CD3, CD5, CD10, CD30, CD56, CD117, ALK, bcl-2, bcl-6 and MPO. These vessels also stained positive for ERG (Fig. 2d) and negative for podoplanin. The patient was diagnosed with intravascular large B-cell lymphoma and initiated rituximab-based chemotherapy and intrathecal methotrexate. Diagnosing intravascular large B-cell lymphoma is challenging as cutaneous signs are protean and can mimic other inflammatory dermatoses. Morphological appearances can include nodules, plaques or macules of varying distributions. Random skin biopsies of clinically unaf-