IgG4-related sclerosing disease is recognized as a distinct clinicopathological entity. It is well known that this disease can occur in the salivary, lacrimal and pituitary glands, in the head and neck region. The nasal cavity is an extremely rare site of involvement of IgG4-related sclerosing disease. Herein is reported a case of multiple IgG4-related sclerosing lesions in the maxillary sinus, parotid gland and nasal septum. A 73-year-old Japanese man presented with nasal obstruction and tumors of the right maxillary sinus and parotid gland were detected, after which resections of these tumors were performed. One year after the last surgery, he noted swelling of the nasal septum, and the tumor was resected. These three tumors had similar histopathology, such as conspicuous fibrosclerotic changes with dense lymphoplasmacytic infiltration and occasional obliterative phlebitis. Immunohistochemistry indicated abundant IgG4-positive plasma cell infiltration and high ratios of IgG4-positive/IgG-positive plasma cells (>70%) in all three lesions. The diagnosis of multiple IgG4-related sclerosing lesions was made. The present case suggests that IgG4-related sclerosing lesion can occur in the maxillary sinus and nasal septum, and represents an extension of the spectrum of IgG4-related sclerosing disease.

IgG4-related sclerosing disease can occur in the cardiovascular system and some inflammatory abdominal aortic aneurysms have been shown to belong to IgG4-related sclerosing disease. Herein is reported a case of IgG4-related inflammatory aortic aneurysm of the aortic arch. A 71-year-old Japanese man was found to have an aneurysm of the aortic arch with maximum dimension of 5.5 cm. The surgically resected aneurysm wall had conspicuous fibrosclerotic changes, dense lymphoplasmacytic infiltration and occasional obliterative phlebitis in the adventitia; the thickness of the adventitia was 6.5 mm. Immunohistochemistry indicated numerous IgG4-positive plasma cell infiltrates; 84% of the IgG-bearing cells were IgG4 positive. The diagnosis of IgG4-related inflammatory aortic aneurysm of the aortic arch was made. Although previously reported IgG4-related inflammatory aortic aneurysms were confined to the abdominal aorta, the present case report demonstrates that IgG4-related inflammatory aortic aneurysm can occur in the aortic arch, thereby extending the spectrum of IgG4-related periaortitis. Further studies are needed to clarify the spectrum of IgG4-related sclerosing disease in the cardiovascular system.

A carcinoma displaying undifferentiated features with dense lymphoplasmacytic infiltration is defined as a lymphoepithelioma-like carcinoma (LEC), and some of LEC is associated with Epstein-Barr virus (EBV). All of the 13 previously reported cases of LEC of the biliary system were intrahepatic in location. Herein, we describe the first case of LEC of the inferior common bile duct. A 68-year-old Japanese man, who had been previously treated for hepatocellular carcinoma using microwave coagulation therapy, was found to have tumors of the common bile duct and pancreas head. Histopathological study of the resected tumor showed solid or cohesive nests of large undifferentiated cells with irregular large vesicular nuclei and nucleoli. Around the tumor cell nests, dense lymphoplasmacytic infiltration was observed. Focal glandular differentiation (approximately 5%) was also present. These histopathological features corresponded morphologically to LEC. Immunohistochemically, the tumor cells were positive for cytokeratin (CK) 7, CK 19 and CA19-9, but negative for CK 20 and Hep Par 1. In situ hybridization for Epstein Barr virus early small RNAs disclosed no nuclear signal in tumor cells. Therefore, a diagnosis of non-EBV-associated LEC of the inferior common bile duct was made. Although the prognosis of the biliary LEC is thought to be better than that of conventional cholangiocarcinoma, the differences in prognosis between EBV-positive and -negative cases have not yet been established. Therefore, additional case studies will be needed to clarify the clinicopathological features of LEC of the biliary tract.

Granulomatous mycosis fungoides (GMF) represents an uncommon variant of mycosis fungoides (MF) characterized by the presence of an associated granulomatous reaction. Most cases of GMF are CD4 positive, and CD8 positive cases are extremely rare. Herein, we report a case of CD8-positive GMF. A 75-year-old Japanese woman presented with brownish maculae on the trunk and upper and lower extremities. She had been diagnosed with MF, and most of the eruption improved by psoralen ultraviolet A therapy. However, the eruption relapsed and gradually expanded 5 months prior to her visit to our hospital. Histopathology showed an atypical lymphocytic infiltrate in the dermis accompanied by granulomatous reaction with multinucleated giant cells. Epidermotropism was evident and elastophagocytosis was also found. Immunohistochemically, the atypical lymphocytes expressed betaF1, CD3 and CD8, and some of the atypical lymphocytes were also T cell intracellular antigen-1 positive. These findings were consistent with CD8-positive GMF. The dermatopathological diagnosis of GMF is challenging in some cases because of the prominent secondary granulomatous reaction. Therefore, when dermatopathologists diagnose granulomatous skin lesions, GMF should also be considered. In addition, the prognosis of GMF, especially CD8-positive GMF, is still controversial. Additional studies are required to clarify the clinicopathological features of CD8-positive GMF.

BackgroundThe clinical characteristics of myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) positive granulomatosis with polyangiitis (GPA) remain unclear, as does the difference between MPO-ANCA positive GPA and proteinase 3 (PR3)-ANCA positive GPA, especially with regard to the details of respiratory tract involvement. We investigated the differences in clinical, radiological, and histopathological features between PR3-ANCA positive GPA and MPO-ANCA positive GPA.MethodsWe retrospectively reviewed 16 patients who were newly diagnosed with GPA between December 2000 and July 2014. One patient, who was positive for both PR3-ANCA and MPO-ANCA, was excluded. Our review was based on the European Medicine Agency (EMA) algorithm.ResultsFifty-six percent of GPA patients were positive for PR3-ANCA, 38 % for MPO-ANCA, and the remaining 6 % for both. The MPO-ANCA positive group included a greater number of females (67 %). There were no statistically significant differences in laboratory data, symptoms and signs, Birmingham Vasculitis Activity Score, or CT findings between the two groups. As for upper respiratory tract involvement, the most common manifestation was paranasal sinusitis, whereas lung nodules were most common as the lower respiratory tract involvement in both groups. Although the combination therapy with prednisone and cyclophosphamide was the most common initial treatment in both groups, the relapse rate in MPO-ANCA positive cases was lower than that of PR3-ANCA positive cases (17 % and 56 %, respectively).ConclusionA high prevalence of MPO-ANCA positive GPA was noted. No significant differences in clinico-radiological findings were observed except for the prevalence of relapse between the PR3-ANCA positive cases and MPO-ANCA positive cases, suggesting that the type of ANCA may be of little help in the diagnosis of GPA. Examination for granulomatous findings in the respiratory tract is important, even in MPO-ANCA positive cases. There is a need to accumulate more cases and conduct a further investigation in the future.