Fumaric acid esters in the treatment of psoriasis: an Italian experience.

BACKGROUND: Psoriasis is a common, chronic, cell-mediated, inflammatory skin disease. Treatment limitations and a developing understanding of its pathogenesis on a molecular level have encouraged much interest in the field of immunomodulatory therapy. OBJECTIVE: To evaluate the efficacy and safety of fumaric acid esters, in particular dimethylfumarate (DMF), in the treatment of moderate to severe plaque psoriasis intolerant and/or resistant to other conventional systemic therapies. METHODS: A total of 40 patients were enrolled in this study. DMF was orally administered at the daily dose of 30 mg up to 360 mg for a minimum of 6 months treatment. Patients were followed-up with psoriasis area and severity index (PASI) score assessment, and clinical and photographic documentation. RESULTS: A total of 33 (82.5%) patients achieved complete clinical remission with DMF treatment: eight after 3 months and 25 after 6 months. Adverse events, such as intolerable abdominal cramps and incoercible diarrhoea, occurred in four patients who, for this reason, interrupted therapy. CONCLUSION: The findings suggest that DMF is a safe, effective and well-tolerated long-term oral treatment worthy of consideration for selective patients.

Treatment of vitiligo with the 308 nm excimer laser.

Several therapeutic modalities have been proposed for the treatment of vitiligo either to achive repigmentation in the lesions or to stabilize the disease. Narrow-band UVB therapy has been shown to be effective and safe for use in the management of vitiligo; its wavelength is not so different from 308 nm XeCl excimer laser radiation. We present an open and uncontrolled pilot study of 24 patients (12 men, 12 women) in whom vitiligous patches were treated twice a week, for 9 months with 308 nm XeCl laser radiation. Seven of the 24 patients showed greater than 75% repigmentation, six patients showed repigmentation of between 25 and 75% and six patients showed less than 25% repigmentation. In five patients no signs of repigmentation were noted. The therapeutic benefit was stable during the 12-month follow-up period. Although these results are promising, treatment has so far been limited to small numbers of patients and a short follow-up period. Other prospective studies are needed to assess the efficacy of this treatment modality.

Successful treatment of multiple filiform facial warts with imiquimod 5% cream in a patient infected by human immunodeficiency virus.

Imiquimod, an imidazoquinoline amine, is approved for the topical treatment of external anogenital warts induced by human papilloma virus. Several clinical trials have shown imiquimod to be an effective and safe drug for treatment of anogenital warts. Consequently, it was considered that imiquimod might be effective on warts caused by the same aetiological agent located on other skin areas. We describe the favourable outcome of a case of multiple facial verrucae in a human immunodeficiency virus (HIV)-infected patient treated with imiquimod 5% cream. This is a promising finding which supports those of two previous reports. We feel that imiquimod could be used in HIV-infected patients with multiple facial warts in whom conventional therapies are ineffective or produce significant side-effects.

Diagnosis of pigmented skin lesions by dermoscopy: web-based training improves diagnostic performance of non-experts.

BACKGROUND: Dermoscopy has been shown to enhance the diagnosis of melanoma. However, use of dermoscopy requires training and expertise to be effective. OBJECTIVES: To determine whether an Internet-based course is a suitable tool in teaching dermoscopy, and to evaluate the diagnostic value of pattern analysis and diagnostic algorithms in colleagues not yet familiar with this technique. METHODS: Sixteen colleagues who were not experts in dermoscopy were asked to evaluate the dermoscopic images of 20 pigmented skin lesions using different diagnostic methods (i.e. pattern analysis, ABCD rule, seven-point checklist and Menzies' method), before and after an Internet-based training course on dermoscopy. Mean +/- SEM sensitivity, specificity and diagnostic accuracy, and kappa (kappa) intraobserver agreement were evaluated for each diagnostic method before and after training for the 16 participants. Differences between mean values were assessed by means of two-tailed Wilcoxon rank-sum tests. RESULTS: There was a considerable improvement in the dermoscopic melanoma diagnosis after the Web-based training vs. before. Improvements in sensitivity and diagnostic accuracy were significant for the ABCD rule and Menzies' method. Improvements in sensitivity were also significant for pattern analysis, whereas the sensitivity values were high for the seven-point checklist in evaluations both before and after training. No significant difference was found for specificity before and after training for any method. There was a significant improvement in the kappa intraobserver agreement after training for pattern analysis and the ABCD rule. For the seven-point checklist and Menzies' method there was already good agreement before training, with no significant improvement after training. CONCLUSIONS: We demonstrated that Web-based training is an effective tool for teaching dermoscopy.

Epstein-Barr virus associated diffuse large B-cell lymphoma complicated by autoimmune hemolytic anemia and pure red cell aplasia.

A 53-year old man with systemic lymphadenopathy and hepatosplenomegaly was diagnosed with diffuse large B cell-lymphoma after inguinal lymph node biopsy. Anemia was noted, direct and indirect Coombs tests were positive, and the haptoglobin level was low. However, the bone marrow aspirate revealed erythroid aplasia. Co-existing autoimmune haemolytic anemia (AIHA) and pure red cell aplasia (PRCA) were diagnosed. In situ hybridization with Epstein-Barr virus (EBV) encoded small RNA (EBER) showed positive findings in lymphoma cells. Southern blot hybridization revealed immunoglobulin heavy chain gene rearrangement and a clonal EBV terminal repeat, indicating monoclonal proliferation of EBV in infected B cells. The patient was treated with CHOP, resulting in a complete remission (CR). AIHA and PRCA subsided after 3 courses of chemotherapy. In conclusion, this case demonstrates not only the association of B-cell lymphoma with autoimmune disorders but also the involvement of EBV in these conditions.

[Churg-Strauss syndrome (AGA) in Japan].

Churg-Strauss syndrome (CSS) is a rare disease belonging to the group of necrotizing vasculitis. With introduction of guideline therapy for bronchial asthma, many patients have enjoyed the benefit regardless their etiology of asthma. Recently, however, there have been multiple case reports of CSS with relation to leukotriene modifiers, leading to a concern of the potential risk association with the development of CSS and the treatment. We have analyzed recent 16 cases of CSS in our group. Twelve cases had been prescribed steroids (7 oral steroid and 5 inhaled steroid) for a treatment of asthma. Three cases had taken leukotriene modifiers. But two of them were under steroid reduction course. It remains unclear what the exact mechanism of this syndrome is, but physicians must be wary for the signs of CSS, particularly in patients with asthma in whom corticosteroids are tapered.

A study of clinical features of cough variant asthma.

Patients with cough variant asthma (CVA) and classic asthma are frequently among subjects who present at clinics complaining of a chronic persistent cough. To reveal the features of CVA, we examined the differences in the clinical appearance between CVA and classic asthma. Ten CVA subjects and 11 classic asthmatics were enrolled in the study; they were recruited among patients who presented at the National Minamiokayama Hospital complaining of a chronic cough. The number of eosinophils in peripheral blood was 256 +/- 45.8/microl in CVA and 400 +/- 123/microl in classic asthma. Eosinophils represented 67% of the cells of sputum in CVA and 82% in classic asthma. Bronchial responsiveness to methacholine was Dmin 1.37 +/- 0.56 U in CVA and 0.71 +/- 0.46 U in classic asthma. There was no significant difference in these three parameters. There was only a significant difference in V25 between CVA and classic asthma, 80.0 +/- 6.9 and 52.2 +/- 10.0%, respectively. Eosinophil inflammation was almost the same in both CVA and classic asthma.

[Pulmonary involvement, pleural effusion, and electrocardiographic abnormality in hypereosinophilic syndrome].

A 47-year-old man was admitted to our hospital with complaints of cough and shortness of breath. Chest radiography showed infiltration of the right lung and left pleural effusion, the eosinophil count increased notably in the peripheral blood, sputum, and pleural effusion. Transbronchial lung biopsy revealed the invasion of eosinophils like eosinophilic pneumonia. Heart failure easily developed in this patient after the intravenous infusion. Myocardial involvement was suspected, and hypereosinophilic syndrome was diagnosed. After prednisolone was administered, the peripheral blood eosinophil count normalized rapidly, and subsequently, the pleural effusion and infiltration shadows in the lung disappeared.

[Improvement of a case of broncholithiasis after spontaneous lithoptysis].

A 53-year-old woman was admitted for recurrent hemoptysis and cough. The chest radiograph showed an infiltrative shadow in the left upper region. Chest tomogram and CT scan showed a small calcification and consolidation in the left upper lobe. Fiberoptic bronchoscopy revealed fresh hemorrhage from the left upper bronchus but no broncholith or bleeding point were detected. Since the symptoms had disappeared by 10 days after admission, the patient was discharged and followed up as an outpatient. Three weeks later, she spontaneously expectorated a stone 3 mm in maximum diameter, with an irregular surface. Analysis revealed that the stone's composition was 56% of calcium phosphate and 44% of calcium carbonate. Hemoptysis seemed to have been caused by the broncholith, which had originated as a calcification of a peribronchial lymph node that subsequently eroded its way into the airway. After lithoptysis, no recurrence has been observed.

[A case of elderly patient with pulmonary tuberculosis considered to be caused by exogenous reinfection].

A 84-year-old woman presented with chronic febrile illness and anorexia from June 1998. She was diagnosed as pulmonary tuberculosis and was admitted to our hospital in August 1998. Her sputum smear was Gaffky 2, and the type of chest radiograph was b III 3. By family contact examination in August 1998, chest radiological examinations of her husband, a 86-year-old man, showed consolidation in middle lobe, right pleural effusion and two calcified mediastinal lymphnodes. He was diagnosed as pulmonary tuberculosis complicated with pleurisy. He had poor controlled diabetes mellitus. Tubercle bacilli isolated from their sputa showed the same pattern in restriction fragment length polymorphism analysis. Pulmonary tuberculosis of the husband was considered to be caused by exogenous reinfection.

[Serum soluble interleukin-2 receptor in patients with pulmonary mycobacterial diseases].

Serum soluble interleukin-2 receptor (sIL-2R) levels were measured in patients with untreated pulmonary tuberculosis (24 cases), patients with multidrug-resistant intractable pulmonary tuberculosis (7 cases) and patients with pulmonary non-tuberculous mycobacteriosis (27 cases). Serum sIL-2R levels were elevated in patients with pulmonary mycobacterial diseases and were elevated in untreated pulmonary tuberculosis patients than in other patients. In patients with new tuberculosis, serum sIL-2R levels were higher in patients with extensive lesions. Serum sIL-2R level showed significant positive correlation with serum C-reactive protein level and erythrocyte sedimentation rate, and significant negative correlation with serum albumin level. In patients with intractable tuberculosis and patients with non-tuberculous mycobacteriosis, serum sIL-2R levels were lower than in patients with new tuberculosis. Even in patients with extensive lesions, serum sIL-2R levels were not elevated. Lower levels of serum sIL-2R, marker of immunocompetent cell activity, suggested that immunocompetent cell activity was suppressed in intractable tuberculosis and in non-tuberculous mycobacteriosis.

Influence of age and gender on iodine-123 MIBG kinetics in normal lung.

UNLABELLED: Iodine-123 MIBG is a biochemical marker that can be used to monitor pulmonary norepinephrine (NE) metabolism. The purpose of this study was to characterize pulmonary I-123 MIBG kinetics in relation to age and gender. MATERIALS AND METHODS: Seventeen healthy volunteers and 14 patients with no cardiac or pulmonary disorders were included in this study (age range: 24 to 88 years, mean age 50.2 +/- 17.6 years; 16 males, 15 females). Planar images were obtained 15 min (early) and 3 h (delayed) after injection of I-123 MIBG (111 MBq). Pulmonary uptake of I-123 MIBG was quantified based on the lung-to-mediastinum ratio (LMR) on early and delayed images. The lung clearance rate (LCR) was calculated form both the early and delayed images. RESULTS: Both early and delayed LMR values increased slightly, although they showed no significant correlations with age. There was a significant inverse correlation between LCR and age (r = -0.57, p < 0.001). Neither LCR nor LMR differed significantly between male and female patients, but the mean age of the men was lower than that of the women. CONCLUSIONS: Pulmonary I-123 MIBG kinetics may reflect age-dependent changes in NE metabolism. The effects of age should be taken into account when assessing pulmonary NE metabolism with I-123 MIBG.

[Successful treatment for advanced and refractory chronic lymphocytic leukemia with fludarabine phosphate].

53-year-old man with chronic lymphocytic leukemia resistant to alkylating agent containing regimens, was treated with fludarabine phosphate. Hematological data before the administration of fludarabine phosphate was as follows: RBC 216 x 10(4)/microliter, Hb 7.7 g/dl, WBC 69,400/microliter (lymphocyte 96%), PLT 0.2 x 10(4)/microliter. Bone marrow was fully occupied with lymphocytes. Red blood cells and platelets transfusions were frequently required. Fludarabine phosphate was administered at a dose of 40 mg (23 mg/m2) intravenously for 5 days every 4 weeks. After the start of the therapy, peripheral lymphocyte counts were markedly decreased and recovery of normal hematopoiesis was observed in bone marrow. Any transfusions were no longer necessary. Fludarabine phosphate may be active even for advanced, refractory and terminal stage chronic lymphocytic leukemia.