Fulminant rhabdomyolysis in a patient with dermatomyositis

We describe the clinical and pathologic features of a patient with dermatomyositis presenting as a sudden, rapidly worsening condition leading to widespread muscle destruction and death. Case report. A 25-year-old woman presented to Henry Ford Hospital with a 1-week history of proximal muscle weakness, myalgias, low-grade fever, and an erythematous rash involving the face and trunk. Medical history was not contributory, and the patient denied alcohol or drug use, exposure to toxins, or recent travel. At admission, the skin rash involved the face in a butterfly distribution and was accompanied by bluish-red plaques on the knuckles (Gottron's sign). There was moderate proximal weakness in the upper and lower limbs without atrophy, fasciculations, or Babinski sign. Sensorial and mental examinations were unremarkable. Serum CK was 5,034 UA. EMG revealed myopathic findings. The patient was admitted to the hospital with a provisional diagnosis of dermatomyositis and was started on prednisone 1 mg/kg/d. Shortly after admission, a muscle biopsy of the left quadriceps showed only a few necrotic fibers with no inflammatory infiltrate. The patient became progressively weaker, with increased tenderness and inability to raise the extremities or the head above the level of the bed. Intravenous therapy with methotrexate 0.5 mg/kg/wk and, 2 days later, plasmapheresis were initiated. However, serum CK continued to increase, reaching values of 189,000 U/l by the 10th hospital day. The patient developed myoglobinuria and renal failure, requiring hemodialysis. A second muscle biopsy, taken from the left deltoid, revealed complete coagulation necrosis of all muscle fibers. On the 12th day of hospitalization, the patient had an episode of cardiopulmonary arrest and remained comatose until death, 10 days later. Pathologic findings. At the time of autopsy, the kidneys showed acute tubular necrosis and numerous casts of myoglobin in the collecting ducts. The psoas, quadriceps, deltoid, biceps, and diaphragm muscles showed widespread ischemic necrosis of practically all skeletal fibers, with coagulation of the sarcoplasm and complete loss of cross striations and nuclei, but no inflammatory infiltrates (figure, top). A marked reduction in the number of endomysial small vessels, especially capillaries, was also readily evident. Immunohistochemical study of the first muscle biopsy revealed deposits of C5b-9 (the terminal complement complex, membrane attack complex) in some of the endomysial vessels (figure, middle). The second biopsy displayed complement deposits in most endomysial and perimysial vessels (figure, bottom), very few T4 (helper) lymphocytes, and no T8 (suppressor/cytotoxic), B, or natural killer lymphocytes. Complement staining was also detected in many of the necrotic fibers. The major histocompatibility complex (MHC) class I antigen was detected in regenerating muscle fibers, endothelial cells, and macrophages. Class I1 MHC antigen was found in endothelial cells but not in muscle fibers. Deposits of complement in blood vessels were not found in tissues other than skeletal muscle. Discussion. Rhabdomyolysis, skeletal muscle fiber injury associated with the escape of cell contents into the extracellular fluid,' rarely occurs with inflammatory myopathies. In a review of 87 patients with rhabdomyolysis, Gabow et all identified alcohol ingestion, muscle compression, and generalized seizures as the most common causes. None of their patients developed rhabdomyolysis in association with an inflammatory myopathy. We are aware of only one other instance of a patient with fatal rhabdomyolysis secondary to dermatomyositis, confirmed by autopsy study: in 1974, Kessler et aI2 reported a 34-year-old woman with dermatomyositis who developed extensive muscle destruction and myoglobinuric renal failure, and died 2 months after diagFigure. (Top) Biopsy of the left deltoid muscle shows rhabdomyolysis, characterized by complete loss of cross striations and nuclei and fragmentation of the sarcoplasm. Hematoxylin-eosin, X200 before 16% reduction. (Middle) The first biopsy shows complement deposits in the endomysial vessels. The muscle fibers show no evidence of necrosis. Immunoperoxidase stain with counterstain, x400 before 16% reduction. (Bottom) Strong staining for complement in perimysial vessels of left deltoid muscle. Immunoperoxidase stain, X400 before 16% reduction.