Paraneoplastic dermatomyositis associated with gallbladder carcinoma: a case report and mini-review of the published work.

Sir,

Paraneoplastic dermatomyositis (PDM) associated with gallbladder carcinoma (GBC) is extremely rare. We report a case of PDM associated with GBC and describe the clinical characteristics based on a review of English-language published work.[1234]

A 90-year-old Japanese woman was referred to our department for inpatient hospital care for eruptions and progressive muscle weakness that had been present for 1 month and 10 days, respectively. Clinical examination showed characteristic dermatomyositis (DM) eruption with heliotrope rash, erythema keratodes, and Gottron's papules [Figures 1a and b]. Histopathology of the erythema was consistent with DM [Figure 1c]. Abnormal laboratory examination included liver and biliary tract dysfunctions (aspartate aminotransferase, 474 U/L; normal range, 12-35; alanine aminotransferase, 195 U/L; normal range, 6-33; gamma-glutamyl transpeptidase, 98 U/L; normal range, 3-54) and elevated serum C-reactive protein (2.75 mg/dl; normal range, 0-0.2) and creatine kinase (CK) (7811 U/L; normal range, 36-177 U/L) levels. Autoantibody screening was positive for antinuclear antibody (titer, 1:80; normal, <1:40) and negative for anti-Jo-1 antibody. Serum tumor markers, including carcinoembryonic antigen and carbohydrate antigen-125, were negative. Electromyography showed a myogenic pattern. Interstitial pneumonia was not seen on computed tomography, but the result was consistent with liver and paraaortic lymph node metastases from GBC [Figure 1d]. PDM associated with GBC was diagnosed. There were no indications for surgery or chemotherapy because she had advanced GBC and was advanced in age. Oral administration of prednisolone 50 mg/day, topical application of 0.1% tacrolimus hydrate on the face and 0.05% difluprednate on the extremities, and muscle functional rehabilitation treatment were started. The eruptions faded in 14 days [Figure 1e and f] and serum CK levels reached normal limits. The prednisone dose was decreased gradually to 35 mg/day. However, the muscle weakness progressed. She was transferred to a palliative care hospital because of further progression of her cancer.

Figure 1

Clinical findings (a, b) at first visit and (e, f) after treatment, (c) histopathological study, and (d) computed tomography scan. (a, b) Edematous erythemas on the forehead and eyelids, and erythema keratodes on the knee were seen. (c) Histopathology of the erythema on the right knee showed a liquefaction degeneration of the basal cell layer and a mild perivascular lymphocytic infiltrate in the superficial dermis. (d) Computed tomography showed a swollen gallbladder, thickening of the gallbladder wall, multiple space occupying lesions of the liver, and paraaortic lymph node swelling. (e, f) The eruptions faded in 14 days

There have been five patients with PDM associated with GBC.[1234] All of these patients were female and the age range was 44-90 years. In all evaluable cases, heliotrope rash and Gottron's papules were seen. In four of five patients, elevated CK levels and positive antinuclear antibody test results were seen. In all evaluable patients, negative anti-Jo-1 antibody test results were seen. One patient showed elevated levels of tumor markers, suggesting that the markers might not have diagnostic reliability. Two patients received surgery for GBC. Three patients received systemic administration of steroids for the treatment of PDM. In all evaluable patients, the eruptions improved while muscle weakness improved in two patients, suggesting that skin response to treatment was better than muscle response. Only one patient is alive with no evidence of cancer recurrence or skin eruption.

This is the first review report of PDM associated with GBC. In our case, the entire eruption regressed, but muscle weakness did not improve. The results may be associated with an underlying malignancy and/or progressive disuse syndrome. The case also illustrates the importance of medical practitioners making efforts to improve the quality of life of patients with poor prognosis.