Clinical experiences of different dosing schedules of rituximab in pemphigus with various disease severities.

BACKGROUND: Rituximab, a monoclonal antibody directed against B lymphocytes, has been found to be a therapeutic agent for severe, refractory autoimmune bullous diseases. However, a dosing schedule or treatment indication of rituximab has not yet been established. OBJECTIVES: We investigated the efficacy of rituximab and different dosing schedules for different disease severities, retrospectively. METHODS: A total of 23 patients with pemphigus who received rituximab were evaluated by a review of medical records. Group 1 patients (n = 10) with severe pemphigus were treated with three or four infusions of rituximab at a dose of 375 mg/m(2) at 1-week intervals. Group 2 (n = 13) patients with mild to moderate pemphigus were treated with two infusions of rituximab at the same dose. Late end points, occurrence of relapse and adverse events and numbers of B cells were evaluated. RESULTS: The mean follow-up period was 25.6 months in group 1 and 17.8 months in group 2. In group 1, six patients (60.0%) achieved complete remission (CR), including two patients off therapy (CR OFF) and four patients on therapy (CR ON). The other four patients (40.0%) achieved partial remission on therapy (PR ON). In group 2, nine patients (69.2%) achieved CR (4 CR OFF, 5 CR ON) and four patients (30.8%) achieved PR ON. During the follow-up period, relapse occurred in five patients of group 1 and three patients of group 2. No serious adverse events were observed in any patients. CONCLUSIONS: We concluded that rituximab is an effective and safe treatment method not only in severe, recalcitrant pemphigus but also in mild to moderate pemphigus. Low dose of rituximab seemed to be sufficient to treat mild to moderate pemphigus.

Skin conditions presenting in emergency room in Korea: an eight-year retrospective analysis.

BACKGROUND: Dermatological patients do visit the emergency department (ED) while most skin problems are controllable in the outpatient clinic. However, there is paucity of data on skin conditions presenting in ED. OBJECTIVE: The aim of this study was to provide the demographic and clinical data on dermatological problems in the ED in a secondary hospital in Korea. METHODS: A retrospective study was conducted based on dermatological international classification of diseases (ICD) rendered in the emergency department of Seoul National University Boramae Hospital, Seoul, Korea, during an 8-year period from 2003 to 2010. Most skin conditions were diagnosed by emergency medicine specialist, not dermatologist. RESULTS: A total of 8332 patients with skin problem were seen corresponding to 3.2% of total ED visits. The number of patients had increased 277% from 621 visits in 2003 to 1719 visits in 2010 with a peak incidence in the summer season annually. Urticaria and angioedema group was most common (68.1%), followed by infections group and nonspecific and descriptive diagnosis group. The mean length of stay was distributed preponderantly around one hour. In 6.2% cases, the patient was admitted, most frequently for infections. CONCLUSION: During the eight-year period, dermatological ED patients increased at a steeper rate than total ED patients. However, the non-urgent dermatologic ED patients have increased with respect to admission rate, death rate, length of stay and visiting-admitting discordance. Health policy makers could utilize these basic data to amend the current health delivery system to reduce unnecessary expenditure of medical resources.

Eruptive pseudo-angiomatosis lesions are associated with intravascular neutrophils and do not harbour Epstein-Barr virus.

BACKGROUND: Eruptive pseudo-angiomatosis (EPA) is a rare, relatively newly described cutaneous disorder characterized by the sudden onset of several bright red, angioma-like papules surrounded by blanched halo. Its aetiology is unknown; however, viral infection or mosquito bites have been speculated as possible causes. OBJECTIVE: This study aims to determine the clinical and histopathological features of EPA, and whether it is associated with Epstein-Barr virus (EBV) infection. METHODS: We conducted a retrospective chart review of 25 EPA cases from 2006 to 2008. In order to determine latent EBV infection, EBV-encoded small RNA (EBER) in situ hybridization was performed in 18 subjects. To determine EPA's distinguishing histological characteristics, we compared the cases with 22 control cases of perivascular lymphocytic infiltration for haematoxylin and eosin, CD3, CD4, CD8, CD31 and c-kit staining patterns. RESULTS: The patient sample's female-to-male ratio was 2.1 : 1, and the patients' age ranged from 5 to 79 years (average 46 years). The lesions appeared during the months of July to September in all but 3 patients. Skin biopsies demonstrated capillary ectasia with perivascular mononuclear cellular infiltrates in the upper dermis. Most patients were otherwise healthy, and routine laboratory results were all normal except in one patient who had diabetes. The skin lesions faded without any treatment in 1-2 weeks. Results of EBER in situ hybridization were all negative. The only histological distinguishing feature of EPA was the presence of intravascular neutrophils, which was found to be present in 19 of the 20 EPA cases (95%), in contrast to only 3 of the 22 control subjects (14%) (P < 0.0001). CONCLUSION: The sudden onset of lesions during the summer months among our patients supports the 'paraviral eruption' concept of this probably underdiagnosed condition. The significant presence of intravascular neutrophils may be a diagnostic clue of EPA in South Korea.