Although the etiology of Behçet’s disease (BD) is poorly understood, the number of BD patients registrated with the Ministry of Health, Labor, and Welfare is still increasing and it reached almost 18,000 in 2002. Epidemiologically, the prevalence rate of BD patients worldwide is estimated to be high in Middle-Eastern countries and the districts along the “Old Silk Road” to China, Korea, and Japan. More than 60% of BD patients are reported to have HLA-B51 in their genetic background. There are some etiological hypotheses for BD, including virus infection, bacterial infectious allergy, and abnormal immunological phenomena including autoimmunities, etc. Recently, however, streptococcal infectious allergy associated with various immunoabnormalities which induce 65kDa of heat shock protein (HSP-65) and HSP-60 in the sera of the patients has been pointed out as an important factor in BD pathogenesis. Herein, I would like to introduce the recent study trends and our experimental results showing that Streptococcus (S.) sanguis (uncommon KTH-1 selotype) DNA gene (Bes-1) was found in the BD lesions (aphthous and genital ulcerations, perifoliculitis and erythema nodosum-like eruptions) by polymerase chain reaction (PCR) method PCR-in situ hybridization (ISH) revealed the presence of Bes-1 in the endothelial cells and macrophages that had infiltrated into the lesions. The results suggest that S. sanguis from the infectious foci of the patients’ oral cavity might play an important role in the pathogenesis of BD.
We performed sentinel node (SN) biopsy for 10 patients with cutaneous melanoma. We identified a total of 3 SNs in 2 of the 4 patients who underwent a blue dye method alone (average 1.5 SN). And 10 SNs were identified in all 6 patients who underwent a combination method using a preoperative lymphoscintigraphy and the blue dye method (average 1.67 SN). Six of 8 patients had a micrometastasis of melanoma in the resected SN. MART-1 is thought to be most specific and sensitive among the antibodies used to detect micrometastases. HMB-45 was negative in 3 of the 6 patients who had a metastatic SN. In a primary tumor from one of these 3 patients, most tumor cells in the dermis were negative for HMB-45. Two patients with a metastatic SN underwent complete lymph node dissection (CLND), and histological examination of the resected LNs revealed no additional metastasis. One of 4 patients with metastatic SN who did not undergo CLND developed lymph node metastasis 29 months later. Thus, we performed SN biopsies successfully using preoperative lymphoscintigraphy together with a vital dye. To identify SNs more accurately, we started to use an intraoperative gamma probe-guided sentinel node detection in addition to other methods.
We report two cases of Pneumocystis carinii pneumonia (PCP) that developed during steroid therapy for collagen disease. A 56-year-old woman was reducing her dose of prednisolone after pulse therapy for interstitial pneumonia with dermatomyositis. Suddenly, she developed high fever and dyspnea. Chest CT revealed bilateral interstitial shadows. The patient was diagnosed with PCP by bronchoalveolar lavage fluid (BALF). Despite treatment, she died of respiratory failure. A 48-year-old man had SLE for 11 years, but was in remission for 5 years. He was taking prednisolone 5~7.5mg/day and cyclophosphamide 50~100mg/day. He was hospitalized with a 39~40°C fever and general fatigue, but no other symptoms. Cultures revealed no infection, but plasma β-D-glucan level increased to 908.4pg/ml, and gallium scintigram showed a large bilateral fluid accumulation. These findings suggested PCP. BALF confirmed this diagnosis. CMV pneumonia was diagnosed by CMV antigenemia assay. Bactramin and ganciclovir were administered. We decreased and stopped administration of these drugs as his β-D-glucan, KL-6, and CMV antigenemia levels decreased. However, drug-related bone marrow suppression developed, and the patient died from DIC. These cases suggest that we consider the possible development of PCP when treating patients with collagen diseases with steroids or immunosuppressive drugs.
We treated a case of systemic lupus erythematosus with Pneumocystis carinii pneumonia. A 34-year-old female needed steroid pulse therapy twice because her SLE worsened. After the second steroid pulse therapy, dyspnea and progressing hypoxemia suddenly appeared. Although the chest X-ray did not show a clear pneumonia shadow, a diffuse interstitial pneumonia shadow was observed on chest CT. Various types of antibiotics and immunoglobulin were used without avail. We suspected Pneumocystis carinii pneumonia, and administerted sulfamethoxazole-trimethoprim. The interstitial pneumonia shadow improved rapidly. In order to determine the cause of this pneumonia, we used PCR with BALF (bronchoalveolar lavage fluid) to detect P. carinii DNA, and diagnosed the disease as Pneumocystis carinii pneumonia. Early diagnosis and the cure of infection seem to be important factors that determine the prognosis in compromised hosts treated with steroids.
A 68-year old male had applied a steroid ointment externally on the face since his infancy as a treatment for atopic dermatitis. Since 1996, diffuse yellow plaques had been present on the lower eyelids and cheeks. Blood tests showed no abnormal findings in lipid metabolism, and epidermal atrophy as well as foam cell accumulation from the upper dermis to the middle dermis was observed a histopathological appearance. From these findings, the patient was diagnosed with normolipidemic plane xanthoma. The steroid ointment was stopped and white petrolatum was externally applied with instructions for light protection. However, the xanthoma did not improved. In 1999, we used tacrolimus ointment, and the xanthoma completely disappeared within about one year. Apparently the tacrolimus ointment which has an anti-inflammatory effect equivalent to that of steroid ointments, but does not cause skin disorders such as skin atrophy, or teleangiectasia, was effective.