Rapidly progressive digital necrosis in polyarteritis nodosa

The patient was a 76-year-old woman who started experiencing pain in her thighs, lower legs and feet 2 weeks prior.Subsequently she had fever and experienced difficulties in moving her body.When the patient was referred to our hospital, the second to fifth fingers at or distal to the DIP joints of both hands became black with necrosis, whereas those from the DIP to the PIP joints became dark violet (Fig. 1).Livedo was observed in both thigh regions and the peripheral pulses were not palpable.She was a past smoker, and tests for ANA, RF, ACPA, aPL antibodies, MPO-ANCA and PR3-ANCA were negative.Skin biopsy from an area of the livedo and muscle biopsy were performed.Histopathological examination revealed mediumvessel vasculitis with fibrinoid necrosis and neutrophil infiltration of the skin and blood vessels of the muscle layer, leading to a diagnosis of polyarteritis nodosa.Initial treatment was administered with prednisolone and intravenous cyclophosphamide.In addition, it should be kept in mind that the dark violet skin region before treatment indicates progression of ischaemia, raising the possibility that the region might be necrotized even after starting treatment [1,2].Figure 1.The second to fifth fingers of both hands developed necrosis, turning black distal to the DIP joints