Digital infarcts showing microangiopathy in adult dermatomyositis suggest severe pulmonary involvement and poor prognosis

after the chemotherapy. Raynaud’s phenomenon and digital pitting scars were seen in two and one cases, respectively. The ANA was positive in three of the patients, although no case had scleroderma-specific autoantibodies. In seven patients, skin sclerosis was limited to the extremities, while only one showed skin sclerosis of the trunk as well as the extremities. The atypical distribution of skin involvement without sclerodactyly was specific for our case. Two cases showed morphoea-like plaques. None of the patients had a visceral involvement. Eight of nine cases had minimal persistent scleroderma after withdrawal of the drugs and additional treatments, including oral corticosteroids. Thus, the small incidence of Raynaud’s phenomenon, digital pitting scars and ANA, and the absence of visceral involvement are characteristics of bleomycinor peplomycin-induced scleroderma. Although the pathogenesis of bleomycinor peplomycininduced pulmonary and dermal fibrosis is still unknown, numerous examinations have disclosed several aspects of its mechanism. In normal human dermal fibroblasts, bleomycin enhances the synthesis of type I collagen. In rat lung fibroblasts, bleomycin stimulates pro-a1(I) collagen promoter through the transforming growth factor (TGF)-b response element. Bleomycin or peplomycin enhances reactive oxygen intermediate (ROI) generation from macrophages and polymorphonuclear leucocytes, which provokes inflammation, resulting in tissue fibrosis. Bleomycin or peplomycin induces cytokines such as interleukin-1b and TGF-b, which activate macrophages and polymorphonuclear leucocytes to produce ROI and stimulate dermal fibroblasts to generate collagen, respectively. In addition, cutaneous collagenolytic activity is decreased in animal models treated with bleomycin. Moreover, bleomycin is concentrated in the lung and skin in several species because of the very low activity of the bleomycin-inactivating enzyme, bleomycin hydrolase, in these tissues, which clearly explains the organ-specific adverse effects of bleomycin or peplomycin. In summary, we report the first case of peplomycin-induced scleroderma. Considering the widespread use of bleomycin or peplomycin, it is possible that this type of bleomycin or peplomycin complication may occur more frequently than recognized. Therefore, the history of exposure to chemicals or drugs should be investigated in patients with scleroderma.