Patient: Female, 88Final Diagnosis: Adult onset still’s diseaseSymptoms: Fever • rashMedication: —Clinical Procedure: —Specialty: RhaumatologyObjective:Unusual clinical courseBackground:Adult-onset Still’s disease (AOSD) is a rare multi-systemic inflammatory disorder of unknown etiology characterized by spiking fever, characteristic rash, and arthritis. It often associates with high serum ferritin levels.Case Report:An 88-year-old woman had fever of over 39°C without response to extended-spectrum antibiotics for 6 days. She had non-specific erythema with infiltration on her trunk. She had leukocytosis with neutrophilia of 80%, mild hepatic dysfunction, normal level of rheumatoid factor and antinuclear antibody, thrombocytopenia, elevated d-dimer and soluble interleukin2 receptor, extremely high serum ferritin (78 662 ng/mL), and splenomegaly. Although she had no arthritis or specific erythema, we made the diagnosis of AOSD according to Yamaguchi’s criteria with disseminated intravascular coagulation (DIC) and hemophagocytic syndrome (HPS) after ruling out infections, malignancies, or other connective tissue diseases. Twelve percent of AOSD patients have HPS. The mean serum ferritin of AOSD with HPS was reported at 18 179 ng/mL, which supported the diagnosis of AOSD because only a few other diseases could show such extremely high serum ferritin. Although she was treated with prednisolone (30 mg/day), her condition deteriorated and her left pleural effusion increased. Therefore, methylprednisolone 500 mg/day for 3 days was started followed by prednisolone 30 mg/day and immunosuppressive agent (Cyclosporine 50 mg/day), which improved her general condition, elevated C-reactive protein levels, and extremely high serum ferritin levels.Conclusions:We report the case of an elderly patient with severe AOSD, who developed HPS and DIC, whose extremely high serum ferritin level was useful in diagnosis.

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Patient: Male, 50Final Diagnosis: Proliferative fasciitis/myositisSymptoms: Facial swelling • fever • trismusMedication: —Clinical Procedure: BiopsySpecialty: PathologyObjective:Rare diseaseBackground:Proliferative fasciitis/myositis is a benign disease that can be treated conservatively. However, some patients are mistakenly treated surgically because of a misdiagnosis of the condition as a malignant tumor.Case Report:A 50-year-old Japanese man developed swelling in his left cheek 12 days before admission; he developed a fever and trismus 3 days later. He was admitted to our hospital because of worsening of his condition despite treatment with sitafloxacin for 5 days and needle-aspiration drainage. On admission, he had a fever of 38.1°C, swelling in his left cheek spreading to the lower jaw, and several dental caries. Although ceftriaxone and clindamycin were administered for 7 days because an odontogenic infection was suspected, his condition did not improve. T2-weighted magnetic resonance imaging of the facial muscles on Day 5 of hospitalization showed swelling and high-intensity signals in the left masseter, temporalis, and pterygoid muscles. Macroscopic findings from a biopsy of the left temporalis muscle performed on Day 17 showed white and thickened fascia. Histopathological examination revealed fibrous hyperplasia of the fascia, increased fibrous connective tissue between muscle fibers, and infiltration of inflammatory cells, providing not a definite but a compatible diagnosis of proliferative fasciitis/myositis. Beginning on Day 18, the patient’s fever lessened with gradual improvement of his facial swelling and trismus.Conclusions:It is imperative to include proliferative fasciitis/myositis as a possible diagnosis when patients present with facial swelling and trismus of unknown cause.

An 85‐year‐old woman presented with pain and a palpable mass in her left flank. Abdominal computed tomography revealed massive splenomegaly and para‐aortic lymphadenopathies. Bone marrow biopsy showed CD79a, CD20, and bcl‐2‐positive atypical lymphocytes, which led to the diagnosis of splenic marginal zone lymphoma.

Patient: Male, 63-year-old Final Diagnosis: Pancreatic undifferentiated carcinoma with expansive metastatic infiltration of the portal venous system Symptoms: Low back pain Medication:— Clinical Procedure: — Specialty: Oncology Objective: Challenging differential diagnosis Background: The portal vein thrombosis caused by a carcinoma of the pancreas or liver could be a diagnostic challenge. There are some cases which could be diagnosed only by observing changes in patients’ clinical manifestations or imaging studies over time. Case Report: A 63-year-old man experienced exacerbation of chronic low back pain for 1 month, with abdominal distention. He was admitted to our hospital because abdominal ultrasonography and abdominal contrast-enhanced computed tomography performed at another hospital revealed an extensive thrombus in the portal, splenic, and superior mesenteric veins. Anticoagulation therapy with warfarin was started immediately on admission. Although no baseline disease, such as malignancies, coagulopathies, infections, or collagen diseases were revealed during the 12 days of his hospitalization, the thrombus gradually expanded, with the appearance of obstructive jaundice 8 months after admission. He was readmitted at that time for biopsy of intrahepatic tumors detected by abdominal computed tomography, which diagnosed metastasis of a carcinoma of unknown origin. Although chemotherapy was started, he died 11 months after his initial admission to our hospital. Autopsy revealed a tumorous lesion in the pancreatic head with almost complete replacement of the portal venous thrombus with a similar-appearing tumor. We confirmed the diagnosis of portal venous metastatic infiltration by undifferentiated pancreatic carcinoma, using histopathological examinations. Conclusions: It is essential to consider portal venous metastatic infiltration of undiagnosed malignancy inducing local hypercoagulopathy as a possible cause of expansive portal venous thrombosis.