| Abstract | Dermatomiozitis je rijetka idiopatska upalna miopatija karakterizirana patognomičnim kožnim promjenama (heliotropni eritem, periorbitalni edem, Gottronove papule) i slabošću proksimalnih mišića udova. U jedne trećine oboljelih radi se o paraneoplastičkom dermatomiozitisu, a u zapadnjačkim populacijama najčešće opisana podležeća maligna bolest je rak jajnika, dojke, pluća, gušterače, debelog crijeva, kao i non-Hodgkinov limfom. Patogeneza bolesti nije do kraja razjašnjena, ali se smatra da antigeni izraženi na membrani malignih stanica potiču stvaranje autoantitijela koja se potom vežu za mišićna vlakna i uzrokuju miozitis. Osnova liječenja su kortikosteroidi, uz vrlo čest dodatak imunosupresiva. Rak žučnog mjehura spada u rijetke tumore, ali je pojedinačno najčešći tumor bilijarnog sustava. Poznato je više rizičnih čimbenika te postoji nekoliko teorija karcinogeneze. Zbog dugog asimptomatskog tijeka i nepostojanja adekvatnog probira bolest se uglavnom otkriva u lokalno uznapredovaloj i metastatskoj fazi pa je i prognoza loša. U radu se opisuje slučaj 48-godišnje bolesnice u koje je dermatomiozitis inicijalno bio dijagnosticiran kao vaskulitis. Izostao odgovor na kortikosteroidno liječenje je potaknuo kliničara na dodatnu radiološku obradu kojom je otkriven neoperabilni adenokarcinom žučnog mjehura. Kemoterapijsko liječenje je rezultiralo regresijom maligne bolesti, ali je dermatomiozitis bio neovisnog tijeka, s razvojem novih kožnih promjena. Osim prikaza bolesnice, u siječnju 2019. godine izvršeno je pretraživanje PubMed baze podataka MeSH terminima neoplazma žučnog mjehura i dermatomiozitis te je pronađeno ukupno devet prikaza bolesnika (sedam žena uključno s bolesnicom prikazanom u ovom radu i dva muškarca) s dermatomiozitisom i rakom žučnog mjehura. Dvoje bolesnika je bilo podvrgnuto kirurškom zahvatu nakon čega je uslijedila rezolucija kožnih promjena. Troje bolesnika je kemoterapijski liječeno. Iako je u posljednjem desetljeću postignut napredak u razumijevanju obje bolesti, i dalje je potrebna suradnja specijalista raznih područja. U bolesnika s dugogodišnjim rizičnim čimbenicima za rak žučnog mjehura obvezno bi trebalo učiniti UZV abdomena i savjetovanje o profilaktičkoj kolecistektomiji. U bolesnika s dermatomiozitisom ne bi trebalo previdjeti tumore probavnog sustava (žučni mjehur, gušterača, debelo crijevo) te bi minimalno trebalo učiniti UZV abdomena. |
| Abstract (english) | Dermatomyositis is a rare idiopathic inflammatory myopathy characterized by pathognomic cutaneous manifestations (heliotrope rash, periorbital oedema, and Gottron’s papules) and proximal muscle weakness. Approximately one third of the patients experience paraneoplastic dermatomyositis. In Western populations, the most frequent underlying malignant disease is ovarian, breast, lung, pancreatic, colon cancer, as well as non-Hodgkin lymphoma. The pathogenesis of the disease is not completely clarified. However, it is considered that the antigens expressed on the membranes of malignant cells initiate the forming of autoantibodies, which then tie to muscle fibres and cause myositis. Corticosteroids represent the mainstay treatment, with immunosuppressants as a frequent addition. Although it is the most frequent biliary duct cancer, gallbladder cancer is a rare malignant disease. Several risk factors are recognized, as well as several carcinogenesis theories. Due to long asymptomatic course and the absence of adequate selection, the disease is mostly diagnosed in locally advanced and metastatic stage. Therefore, the prognosis is poor. In this paper, the case of a 48-year-old female patient whose dermatomyositis was initially diagnosed as vasculitis is presented. Following the patient's inadequate response to corticosteroid treatment, clinical and radiologic examinations were performed, showing inoperable gallbladder adenocarcinoma. Although initial chemotherapy led to regression, the dermatomyositis developed an independent course with new pathological changes leading to the progression of the disease. Besides presenting the case, in January 2019, PubMed database was searched by using MeSH terms gallbladder neoplasm and dermatomyositis. A total of nine case reports with dermatomyositis and gallbladder cancer were found (seven females, including the patient presented here, and two males). A surgical procedure was undertaken on two patients, after which the resolution of cutaneous manifestations followed. Three patients were given chemotherapy. Although in the last decade a progress in comprehending both diseases was made, the cooperation of specialists in various fields is still necessary. In patients with long-term gall bladder cancer risk factors, abdominal US and consulting on prophylactic cholecystectomy should be obligatory procedures. In patients with dermatomyositis, gastrointestinal (gallbladder, pancreatic, and colon) carcinoma should not be omitted, so US should be performed. |
