History of Dermatomyositis

The first detailed descriptions of patients with rare muscle disease (acute form of myositis) with cutaneous lesions were reported by E. Wagner [1] in 1863 and P. Potain (1875) [2]. These authors introduced to the medical community a new group of IIMs, characterized by the damage of many skeletal muscles and by skin manifestations. These disorders are rare, but increasingly recognized. They have avariety of clinical manifestations, immunological abnormalities and courses, and form a diverse group of diseases with unclear causes. The most common forms of these disorders are dermatomyositis (DM) and polymyositis (PM) [3]. In 1887, Professor Hans Unvericht published a description of a peculiar muscle disease with fatigue and malaise, muscle pain and weakness, swelling of face, and bluish lesions over eyelids [4]. He reported a 27-year-old stonemason, who developed acute onset of weakness, stiffness, and pain in the proximal arm, leg, and back muscles. A week later, diffuse swelling of face and extremities, accompanied by a low-grade evening fevers and a bluish rash over his eyelids, had developed. Over the next few days the patient noticed shortness of breath, thickening of the voice, and dysphagia. After several weeks, the patient died with cyanosis and clinical manifestation of a pulmonary arrest. Autopsy showed fluidfilled lungs and swollen proximal muscles. Histopathological examination showed various stages of degeneration of muscle fibers, and focal round-cell interstitial infiltration of the affected muscles. In 1891, Unvericht reported a second case and gave a name to this disorder — dermatomyositis [5]. Up to the end of the nineteenth century, several authors published independently clinical descriptions of these disorders. Twenty-eight cases had been reported, and the name of the disease was coined variously as myositis universalis acuta, polymyositis, pseudotrichinosis, or DM [6–10]. In the first decade of the twentieth century, Jacoby observed a patient with skin atrophy, edema of the face and eyelids, arthralgias, and weakness of muscles, and reported the case as poikiloderma vascularis atrophicans [11]. In 1916 the first two cases of DM with association of malignancy (i.e., paraneoplastic DM) [12, 13] were published. Stertz [12] reported a patient of DM associated with gastric cancer, and Krenkeleit reported DM associated with breast cancer [13]. Petges and Clejat (1926) reported a case with idiopathic atrophic sclerosis of the skin, subcutaneous calcinosis, and myositis [14]. Gottron [15] in 1930 provided an extensive 2