(A) T2-weighted images through the proximal thigh

(A) T2-weighted images through the proximal thigh. weakness. Intravenous immunoglobulin (IVIG) is usually a second-line therapy for patients with steroid-resistant disorders. A 49-year-old woman who presented with a high-grade squamous intraepithelial lesion and positive human papillomavirus (HPV; genotype 31, 55) found on a routine pap smear performed at the local gynecology clinic was referred to our Dot1L-IN-1 Rabbit polyclonal to ZFAND2B hospital. She was a nonsmoker and was not addicted to alcohol. She had no significant past gynecological history, but her father was positive for gastric cancer. The patient had Dot1L-IN-1 never presented any symptoms or laboratory abnormalities due to rheumatologic or neurologic disease. The diagnosis was determined by punch biopsy of the cervix that revealed cervical intraepithelial neoplasia grade III. This was followed by a loop electrical excision procedure (LEEP) around the cervix. The cervical tissue obtained from the LEEP showed squamous cell carcinoma in situ. Four weeks after diagnosis, she was admitted for total laparoscopic hysterectomy. The tissue biopsy obtained from hysterectomy showed no residual tumor after the cervical conization. One month after discharge, the patient visited the emergency room with myalgia, weakness of the shoulder and pelvic girdle muscle, and cutaneous disease. On examination, she had erythema on the upper eyelid, neck and trunk, facial edema, and Gottrons papules on the fingers (Fig. 1). Serum examination showed high levels of creatine kinase (11,744 U/L) and aldolase (67.7 IU/L). Immunological investigations showed positive antinuclear antibody (1:2,560) with negative anti-JO-1, anti-dsDNA, anti-Smith antibodies, anti-Ro, and anti-La, while complement levels were normal. Magnetic resonance imaging (MRI) of the pelvis showed multifocal high signal areas with enhancement at the gluteus, obturator, quadrates femoris, vastus, and gracilis muscles (Fig. 2A). Electromyography showed increased insertional activities, fibrillation potentials, and positive sharp waves in Dot1L-IN-1 the biceps, deltoid, extensor digitorum communis, gastrocnemius medius, tibialis anterior, and vastus lateralis muscles indicating active myopathy. Skin biopsy from an erythematous lesion on the dorsal hand showed perivascular lymphocytic infiltration. Muscle biopsy from the biceps muscle revealed a perifascicular atrophy pattern (Fig. 2B). Based on the clinical characteristics and laboratory findings, we diagnosed the patient with cervical cancer-associated DM. Open in a separate window Figure 1. Clinical pictures. (A) Edema of the eyelids and diffuse erythematous papules were seen on the forehead, and nasal bridge. (B) Erythema of the neck. Open in a separate window Figure 2. Magnetic resonance imaging and pathological findings. (A) T2-weighted images through the proximal thigh. Increased signal intensity was seen in the gluteus, obturator, quadrates femoris, vastus, and gracilis muscles. (B) Microscopic view (H&E, 100) revealed perifascicular atrophy pattern of biceps muscle. Treatment was initiated with intravenous methylprednisolone (60 mg/day). Dot1L-IN-1 The result was partial remission of skin rash, but extremity weakness on exertion and skeletal muscle pain did not respond to the treatment. During admission, the patient demonstrated progressive extremity weakness and myalgia. The serum creatine kinase level remained elevated (13,001 U/L). Abnormal liver function test results were obtained: elevated aspartate aminotransferase (662 IU/L) and elevated alanine aminotransferase (325 IU/L). A computed tomography (CT) scan of the abdomen showed a round hypodense liver mass (Fig. 3A), but chest CT showed no evidence of thoracic metastasis. Serum tumor markers carcinoembryonic antigen and carbohydrate antigen 19-9 increased to 18.11 ng/mL (reference range, 0 to 5.0) and 190.17 U/mL (reference range, 0 to 39), respectively. Liver biopsy was performed and indicated the presence of metastatic adenocarcinoma, probably originating from the colon. Positron emission tomography (PET) scans showed sigmoid colon cancer with pericolic lymph node metastasis and a lesion in the right lobe of the liver (Fig. 3B and ?and3C).3C). Biopsy taken at colonoscopy revealed the presence of adenocarcinoma of the sigmoid colon. The patient underwent right hepatectomy, cholecystectomy, and laparoscopic anterior resection. Subsequent adjuvant chemotherapy with leucovorin, 5-fluorouracil, and oxaliplatin (FOLFOX6 regimen) was performed for 8 cycles. After recovering from surgery, the patient was discharged with oral steroid agents to be administered at our out-patient rheumatic clinic. The steroid treatment was slowly tapered over 6 months by our rheumatologist. Open in a separate window Figure 3. Computed tomography/positron emission.