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A case of dermatomyositis in an elderly woman, found to have gall bladder adenocarcinoma, is presented. As far as we know this is the first report of an association between the myopathy and this specific malignancy.
A 75 year old white woman was admitted to our department, with a seven month history of proximal muscle weakness, dysphagia, dysphonia, facial erythema, and oedema of the eyelids. Atypical dyspeptic symptoms had been present for the same time period. At that time, an abdominal ultrasound showed, besides multiple gall stones, increased echogenicity of the gall bladder content. Simultaneous routine laboratory tests were normal, except for a minimal increase of serum aspartate aminotransferase (AST) and alanine aminotransferase (ALT) and a creatine kinase (CK) value of 350 IU/ml (upper normal 190). Apparently, that had gone unnoticed, until her admission.
On physical examination, she was found to be a relatively obese elderly woman, in no distress, with normal vital signs. A “heliotrope” rash was present in her eyelids, and Gottron's papules were noticed on the dorsal surface of her metacarpophalangeal and proximal interphalangeal joints bilaterally. Proximal muscle weakness of the neck, shoulder, and pelvic girdles was prominent. The rest of the physical examination was unremarkable.
Routine laboratory tests, including CK, were normal, except for a mild increase of AST and lactate dehydrogenase (LDH). Thyroid function was within the normal range. Serum antinuclear antibodies were positive at a titre of 1/160 with a fine speckled pattern. The rest of the serological profile was normal.
A chest x ray examination was unremarkable. Gastroscopy showed mild gastritis. Abdominal ultrasound was essentially the same as the one performed seven months previously. An electromyogram of the proximal muscles disclosed decreased amplitude and duration of motor unit action potentials and an increased proportion of polyphasic potentials, increased insertional activity and fibrillation. A deltoid muscle biopsy showed typical findings of dermatomyositis.
The patient was treated with methylprednisolone 48 mg daily, and asked to be discharged. We continued the investigations to detect a possible underlying malignancy, during a close follow up. She improved clinically with methylprednisolone, and the steroid dose was gradually tapered. Meanwhile, a bone scan and a mammogram were read as normal. However, an abdominal computed tomography (CT) scan showed thickening of the gall bladder wall and enlargement of the lymph nodes around the portal vein and hepatic artery.
Two months after her initial presentation to us, she was admitted to the surgical department for exploratory laparotomy; her muscle strength had improved, and she had no rash or jaundice, while taking methylprednisolone 16 mg/day. At that time, her alkaline phosphatase and γ-glutamyltransferase (γGT) were markedly raised, by three and eight times normal, respectively. At surgery, cancer of the gall bladder was found, with intrahepatic metastases and involvement of the lymph nodes of the hepatoduodenal ligament; a cholecystectomy was performed. The specimen disclosed a moderately differentiated adenocarcinoma, affecting most of the gall bladder wall, and at its full thickness expanding to the surrounding fatty tissue (fig 1).
Ten weeks after discharge, she was readmitted to the medical service, severely debilitated, with profound muscle weakness and jaundice, nodular hepatomegaly, and oedema. Laboratory tests showed mild anaemia, markedly raised alkaline phosphatase, γGT, and LDH, bilirubin of 548 μmol/l, but normal CK. The patient's already serious condition deteriorated further, she developed a left lower lobe pneumonia and died 10 days after her admission.
A number of studies have suggested an association between inflammatory myopathy and malignancy, which seems stronger with dermatomyositis and weaker with polymyositis.1–4 A temporal relationship has also been suggested, with the diagnosis of malignancy coinciding with, preceding, or following that of myositis, usually within two years,2,3,5 although longer periods of surveillance have been recommended.4
With the exception of gastric and ovarian carcinoma, which seem to more commonly underlie myositis, the spectrum of the various kinds of cancer in patients with inflammatory myopathy is similar to that in the general population.3 However, an association of dermatomyositis with tumours of the biliary tract is extremely rare. According to the available published reports, there are only three cases of dermatomyositis in patients with cholangiocarcinoma (two of them in non-English literature),6–8 and in no patient with gall bladder carcinoma has inflammatory myopathy been described. Furthermore, in a recent excellent population based study9 of 618 patients with dermatomyositis, of whom 198 were found to have cancer, no gall bladder carcinoma was identified. Therefore, our patient seems to be the first report of an association between dermatomyositis and adenocarcinoma of the gall bladder. Her myopathy responded initially to steroids, despite the advanced stage of her cancer, in accordance with some observations, suggesting occasionally independent courses of dermatomyositis and malignancy.10
In conclusion, carcinoma of the gall bladder should be added to the list of malignancies, which dermatomyositis can complicate, as a paraneoplastic process.
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