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2012, vol. 18, br. 1-2, str. 40-44
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Paraneoplastični dermatomiozitis u radu lekara opšte medicine
Paraneoplastic dermatomyositis in the work of general practitioner
aDom zdravlja 'Dr Simo Milošević', Beograd bKlinički centar Srbije, Klinika za infektivne i tropske bolesti, Beograd, Srbija
e-adresa: jelena_stanojevic2000@yahoo.com
Sažetak
Uvod. Dermatomiozitis je sistemsko autoimuno oboljenje koje se odlikuje karakterističnim promenama na koži uz postojanje progresivne slabosti skeletnih mišića. Zajedno sa polimiozitisom pripada grupi inflamatornih miopatija. Incidencija obolevanja je 2-7 obolelih na milion stanovnika. Dermatomiozitis je kod jedne trećine obolelih povezan sa nekom drugom autoimunom bolešću, a u 10-30% odraslih sa malignim oboljenjem. Najčešće neoplazme sa kojima je povezan su karcinom dojke, pluća, ovarijuma, debelog creva, uterusa, kao i različiti limfomi. Prikaz slučaja. Prikazan je slučaj pedesetosmogodišnjeg muškarca koji se javio lekaru zbog promena po koži i izražene progresivne slabosti u mišićima. Bio je afebrilan, žalio se na otežano gutanje. Lična anamneza: diabetes mellitus i status post infarctum myocardii. U porodičnoj anamnezi negira postojanje autoimunih oboljenja ili karcinoma. Promene po koži su bile u vidu lividnog eritema i lividno eritematoznih papula na licu, vratu, presternalno, na ramenima i leđima. Laboratorijske analize su pokazale sledeće: sedimentacija eritrocita (SE) 2,8 mm/h, krvna slika- b.o., aspartat aminotransferaza (AST) 176 IU/L, alanin aminotransferaza (ALT) 74 IU/L, laktatdehidrogenaza (LDH) 725 IU/L, kreatin kinaza (CK) 3364 IU/L. Hospitalizovan je na Klinici za dermatovenerologiju. Započeta je terapija sistemskim kortikosteroidima i imunosupresivnom terapijom. Zbog bola u abdomenu poslat je hirurgu u Urgentni centar. Na učinjenom skeneru abdomena uočena promena suspektna na karcinom debelog creva. Preveden je na hirurgiju, na otpustu promene na koži u regresiji uz ponovno pogoršanje mišićne slabosti. Na Prvoj hirurškoj klinici tokom operacije je preminuo. Zaključak. Prikazan je tipičan slučaj paraneoplastičnog dermatomiozitisa. Klasična klinička slika uz jasno povišenje vrednosti mišićnih enzima u serumu. Bez obzira na primenjenu terapiju bolest je imala loš ishod zbog nekoliko faktora: karcinom se pojavio istovremeno sa pojavom dermatomiozitisa, pacijent je imao otežano gutanje a autoantitela su bila negativna, što sve prejudicira loš ishod oboljenja. Neophodno je pažljivo praćenje ovakvih pacijenata od strane lekara opšte medicine.
Abstract
Introduction. Dermatomyositis is a systemic autoimmune disease characterized by skin manifestations associated with progressive weakness of skeletal muscles. Term Inflammatory myopathy encompasses dermatomyositis and polymyositis. The incidence of the disease is 2-7 patients per million. In 1/3 of the patients dermatomyositis is associated with some other form of autoimmune disorder, and in 10-30% adults it is associated with malignancy. The most common cancers associated with dermatomyositis are breast cancer, lung cancer, ovarian cancer, colon cancer, uterine cancer, and different kinds of lymphoma. Case report. This case report is about fifty-eight year old man with complaints of skin changes and strong progressive muscle weakness at presentation. He didn't have a fever but complained of difficult swallowing. Personal history reveals diabetes and acute myocardial infarction. Family history is negative for autoimmune disorders and cancer. Skin lesions were in the form of livid erythema and livid erythematic papules on face, neck, presternal region, on shoulders and back. Laboratory tests revealed: ESR 2.8mm/h, normal CBC, AST 176 IU/L, ALT 74 IU/L, LDH 725 IU/L, CK 3364 IU/L. He was admitted in Clinic of dermatology and treated with systemic corticosteroids and immunosuppressants. Due to abdominal pain the patient was surgically evaluated in the emergency center, where CT scan revealed the mass suspected of colon cancer. He was transferred to surgery ward, skin manifestation were in remission but the muscle weakness started to deteriorate again. During the operation the patient has died. Conclusion. Hereby, a typical case of the paraneoplastic dermatomyositis is presented. Classical clinical presentation with obvious elevation of the muscle enzymes in serum. Regardless the given therapy, the disease had lethal outcome due to several reasons: cancer was diagnosed at the same time as dermatomyositis, the patient had swallowing problems and auto antibodies were all negative, which implies bad outcome. Therefore constant monitoring of these patients is required.
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