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Case of bleomycin‐induced scleroderma

MRSS-3 in three patients. Four patients had ulcers on their feet, and the other six patients had no ulcers. The relationship between the total skin score and SPP, ABI and PWV is shown in Figure 1(a). The patients with MRSS-2 and MRSS-3 scores had significantly lower SPP than healthy controls (P < 0.01). Similarly, MRSS-2 and MRSS-3 patients had significantly higher PWV than healthy controls (P < 0.05). These scores were not significantly different between the MRSS-2 and MRSS-3 groups. ABI was not significantly related to the total skin score. The relationship between skin ulcer and SPP, ABI and PWV is shown in Figure 1(b). The patients with ulcers on their feet had significantly lower SPP and ABI values than those without ulcers (P < 0.05). The PWV value was not significantly related to the presence of skin ulcers. The relationship among SPP, ABI and PWV is shown in Figure 1(c) for the healthy controls and SSc patient groups. In the healthy controls, ABI, PWV and SPP were highly correlated with each other (r = 0.896, r = 0.896, r = 0.836). In the SSc patient group, ABI PWV and SPP were not correlated with each other (r = 0.173, r = 0.069, r = 0.395). Various sizes of vessels are affected in systemic sclerosis. This might cause the differences among healthy controls and SSc patients. However, the study was limited because of the age difference between controls and patients. Compared with ASO, SSc involves smaller peripheral vessels. Therefore, ABI, which is calculated between the upper extremities and under the lower extremities, is not sufficient for evaluating SSc, even though it is useful for evaluating ASO. The total skin score was not related to ABI. The presence of skin ulcers was related to ABI, probably because patients who have digital ulcers might have macrovascular involvement. PWV, which checks the pulse wave of a peripheral artery, was effective for evaluating the advancement of SSc. SPP, which directly measures skin perfusion, is effective for evaluating SSc because direct skin perfusion has few biases and accurately measures skin ischemia. Kanetaka et al. reported SPP decreases when Raynaud’s phenomenon occurs. The present report provides evidence that SPP is useful for evaluating SSc-related ischemia. Generally, surgical therapy is not applied for SSc ischemic digital ulcer due to the risk of ulcer magnification. In ASO, low SPP leads to a low healing rate. It is thought that low SPP would also lead to a low healing rate in SSc. Moreover, SSc causes vasospasm, and is usually treated conservatively with vasodilators and antiplatelet drugs. Recently, endothelin receptor antagonists such as bosentan were shown to be effective for ischemic ulcers caused by SSc. It is not yet clear, however, when treatment with these drugs should be started. There are no guidelines for pharmacological treatment of ischemic ulcer caused by SSc. SPP might provide a standard measure to indicate when to initiate drug therapy, similar to how blood pressure is used for monitoring hypertension.