A 38 year old HIV negative man of mixed ancestry, with a history of smoking, alcohol use, and previously treated pulmonary tuberculosis, presented with severe, constant abdominal pain, arthralgia, swollen hands and skin lesions. Clinically he had marked abdominal pain but no peritonism with discoid type lesions on the scalp as well as auricular ulcers. Raynaud’s phenomenon with digital infarcts of his fingers and toes and a vasculitic rash on his nail beds, palms, soles, and legs, were noted. BP was 155/95 with a tachycardia of 113 bpm. Lower limb weakness was evident with 4/5 globally reduced power demonstrated. Knee and ankle reflexes were reduced. No sensory fall-out was evident. Serum sodium was 128 mmol/L. A diagnosis of a possible acute porphyria, likely variegate, was made. However, the Raynaud’s phenomena, arthralgia, vasculitic rash and digital infarcts were atypical. A Watson-Schwartz test was positive. Urinary ALA and PBG were 68 (upper limit of 1.6) and 79 umol/mmol creatinine (upper limit of 4.5), respectively. Porphyrin plasma fluorescence peaked at 619nm (excitation at 405nm). An R59W PPOX mutation (associated with the South African VP founder effect) was negative. AIP was thus probable, the exact HMBS gene mutation as yet, not identified. Haem arginate was given for 6 days. An autoimmune screen was positive for an antinuclear factor, ANF 1:1280; elevated double stranded DNA 149IU/ml (normal <10IU/ml); elevated total IgG 30.8g/L (normal <16g/L) and low complement C3 and C4. Proteinuria was present (0.8g/day) and serum creatinine normal (65 umol/L). Renal biopsy demonstrated ISN class II Lupus Nephritis. Skin biopsy of the vasculitic lesions confirmed small vessel vasculitis. An SLE diagnosis was made from positive criteria present. Prednisone, Azathioprine and Chloroquine were initiated. Urinary porphyrin activity and acute porphyria symptoms were monitored for. He was discharged a month after admission. Coexisting acute porphyria and SLE is rare. A handful of cases are reported in the literature with most reported cases occurring in women. The putative mechanisms for concomitance invariably are medications used in SLE. However, in our patient, the SLE therapy followed the porphyria presentation, there was a history of alcohol use as well as previous TB treatment with no clinical expression of porphyria. This may suggest an evocative effect of the SLE. In established SLE, triggers of SLE flares often include infections, often viral, physiological, emotional or traumatic stress. How lupus could trigger acute porphyria is unclear, however a ‘perfect storm’ of multiple factors – infection, marked inflammation, carbohydrate depletion may all coalesce to trigger an acute attack. The issue of whether SLE was potentiated by acute porphyria, is a moot point. In summary, we present a case of a man with a rare co-existing presentation of SLE and acute porphyria. The intertwining mechanisms remain unclear.