engleski

Dermatomyositis: a case report

INTRODUCTION Dermatomyositis is an idiopathic inflammatory myopathy of probably autoimmune etiology affecting striated muscle accompanied by characteristic cutaneous findings. Humoral autoimmune response with complement activation and microvasculature damage is the base of the disease. With microangiopathies, migration of activated inflammatory cells to perimysium and endomysium also occurs, resulting in perifascicular muscle fiber atrophy. Symmetric proximal muscle weakness dominates the symptoms, accompanied by skin lesions (heliotrope rash, shawl sign, Gottrons sign). In elderly patients malignant disease incidence is increased. Glucocorticoids are the treatment of choice, with cytostatics, if needed. CASE REPORT A 26-year old female patient was admitted to a county hospital due to muscle pain, especially proximal arm muscles and leg muscles, with muscle weakness, general weakness, and movement difficulty. Muscle weakness was accompanied by pain and swelling of the PIP joints, knees, and joints of the foot. Fever was also present (38, 2°C). During hospitalization macular skin lesions of the face and upper chest accompanied by urticaria-like lesions. Laboratory tests showed a high increase in muscle enzymes (CK 30 780 U/L, CK-MB U/L, LDH 1138 U/L, AST 845 U/L, ALT 387 U/L), elevated CRP 55, 4 mg/L, with normal creatinin level (59 µ mol/L) and normal urine sediment. Tumour markers (CEA, CA 19-9, CA 15-3, CA 125, AFP) were negative. For further diagnostic tests and treatment the patient was transferred to our Clinic. The patient’ s history revealed anemia and thrombocytopenia during the last four years (direct and indirect thrombocytic antibodies were negative on several occasions). Four years ago the patient had a miscarriage in her 12th week of pregnancy. Second pregnancy and labour were without complications. Also four years ago fibroadenoma of the left breast was surgically removed. Two months before admission to the hospital the patient had eaten pork meat untested for Trichinella spiralis. Physical examination showed oedema around the ankles, arm and leg muscles pain-sensitivity to palpation and somewhat difficult walk. The rest of the examination was normal. Most of immunological parameters (anti-ds-DNA, pANCA, cANCA, cryoglobulines, anticitrulin antibodies, C3, C4) were within normal values, but ANA and anti Jo1 antibodies (16 U/ml) were positive. Blood muscle enzyme levels were still elevated, especially CK and CK-MM isoenzyme. Electromyoneurography results showed myopathic changes of dominantly proximal muscles, most likely as part of myositis. Broad diagnostic tests were done to exclude possible infectious and paraneoplastic cause of dermatomyositis. Thyreoid hormones were also normal. Muscle biopsy (m. gastrocnemius) was done, and the pathohistologic report confirmed the diagnosis of dermatomyositis. Intravenous 1mg/kg bodyweight glucocorticoide treatment was initiated, followed by gradual clinical improvement as well as the decrease in muscle enzyme blood levels (CK, LDH, AST, ALT, myoglobin). During hospitalization renal function was continuously monitored, the patient was abundantly hydrated, and the alkalic pH of urine was kept. In good physical condition (with CK 750 U/L, myoglobin 1000U/L) the patient was dismissed to home care, and glucocorticoid treatment was continued. One month after dismission from hospital the patient is feeling good, with normal CK and myoglobin blood levels and normal renal function. CONCLUSION Early diagnosis (imunological parameters, muscle biopsy, EMNG) and treatment are very important for a good recovery of the patient. It is also important to monitor renal function and to exclude dermatomyositis as a part of a paraneoplastic syndrome.

dermatomyositis

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