Sjögren’s syndrome (SjS) is an autoimmune exocrinopathy with a frequency of familial occurrence of 2.2% in Japanese. Recent investigations have shown strong associations between SjS and genetic factors encoded by the major histocompatibility complex (MHC). We presented four patients with SjS in one family. A 29-year-old Japanese woman had annular erythema on the face with anti-SS-A, SS-B antibodies positivity. She was diagnosed with primary SjS. Her male infant also had positive anti-SS-A, SS-B antibodies with indurative erythema. He was diagnosed as having neonatal LE. Other members of her family were also affected. Her younger sister suffered from dry mouth and eyes with Raynaud’s phenomenon and arthritis. She had detectable antibodies against anti-SS-A, RF, Scl-70 and RNP antigens. She was diagnosed with rheumatoid arthritis, SjS and systemic sclerosis. Her mother presented positive anti-SS-A and SS-B antibodies with dry mouth and eyes and was diagnosed with primary SjS. Six members of the family were then analyzed for MHC classes. HLA-A11, B39 and DR8 antigens were expressed in all members, but only four of them were diagnosed as SjS/NLE. The development of SjS appears to result from a complex interplay of multiple HLA-linked and non-HLA linked genetic factors.
We performed a clinicopathological study of granular cell tumor (GCT) by reexamining the clinical data from 73 cases (74 lesions). The study included 23 male and 50 female patients. The average age at resection time was 45.4 years. The tumors were mostly located on the trunk (40%). No case had been clinically diagnosed as GCT; dermatofibroma was the most frequent clinical diagnosis. We examined the histological findings in 29 lesions. Epidermal hyperplasia was present in 74% (17/23 lesions), including 3 lesions with pseudoepitheliomatous hyperplasia. Nodular lymphocytic inflammatory infiltrates were seen in 28% (8/29 lesions). Small peripheral nerves surrounded by granular cells and intranuclear pseudo-inclusion bodies were observed respectively in 48% (14/29 lesions) and 83% (24/29 lesions). These findings support the hypothesis that GCT is a neural tumor.
Intersitial lung disease, a common complication of systemic sclerosis, often has a poor prognosis. Recent studies have shown the effectiveness of cyclophosphamide for preventing deterioration of lung function in patients with systemic sclerosis-associated interstitial lung disease. However, these beneficial physiological effects, which are evident after 12 months of therapy, are no longer apparent at 24 months. Therefore, other potentially immunosuppressive agents with sufficiently low toxicity are needed as maintenance therapies. Myzoribine, which selectively inhibits inosine monophosphate dehydrogenase, is an immunosuppressive agent newly developed in Japan that exerts beneficial therapeutic effects on lupus nephritis and rheumatoid arthritis. In the present study, two patients with systemic sclerosis-associated interstitial lung disease were treated with 150 mg of mizoribine once a day after intravenous cyclophosphamide treatment. In both cases, lung function further improved without any adverse reactions, suggesting that mizoribine can possibly provide effective maintenance therapy following intravenous cyclophosphamide treatment.
We report an 81-year-old Japanese man with heparin-induced thrombocytopenia (HIT). He had received thermal burns over 24% of his body surface (SDB-DB), afflicting his face and arms. He was urgently hospitalized in the ICU and managed by central venous catheters and an arterial monitoring line. Small doses of heparin were used to keep the catheters open. On hospital day 11, the patient’s platelet count dropped. A contrast enhanced CT scan showed a thrombus shadow at the pulmonary artery and deep vein thrombosis in the right thigh. Platelets and a therapeutic dose of heparin were administered intravenously. We debrided the necrotic tissue in his ulcers and applied skin grafts. The platelet count failed to rise after these treatments, and the possibility of HIT was considered. When all heparin therapy was stopped on day 43, the platelet count began to rise gradually. The diagnosis of HIT was confirmed by detection of positive anti-heparin-platelet factor 4 complex antibodies in his serum. Because heparin is widely used for a variety of diseases, we should keep in mind that even small doses may evoke undesirable consequences.