Cutaneous lupus erythematosus and its association with systemic lupus erythematosus: A nationwide population-based cohort study in Korea

Although lupus erythematosus is known to be more common among women of color, the study populations in previous reports were predominantly Caucasian and there is scarce information on Asian patients. Therefore, we performed a retrospective study using a nationwide population-based cohort in South Korea. The average annual incidence of cutaneous lupus was 4.36/100 000. Among 634 patients with cutaneous lupus, 20.8% had systemic disease: cutaneous lupus was diagnosed before systemic lupus in 4.26% and after systemic lupus in 8.52%. More female patients than male patients developed systemic lupus erythematosus. The average time to progression to systemic lupus was 1.53 ± 1.46 years.     

Wound,pressure ulcer and burn guidelines – 4: Guidelines for the management of connective tissue disease/vasculitis-associated skin ulcers

The Japanese Dermatological Association prepared guidelines focused on the treatment of skin ulcers associated with connective tissue disease/vasculitis practical in clinical settings of dermatological care. Skin ulcers associated with connective tissue diseases or vasculitis occur on the background of a wide variety of diseases including, typically, systemic sclerosis but also systemic lupus erythematosus (SLE), dermatomyositis, rheumatoid arthritis (RA), various vasculitides and antiphospholipid antibody syndrome (APS). Therefore, in preparing the present guidelines, we considered diagnostic/therapeutic approaches appropriate for each of these disorders to be necessary and developed algorithms and clinical questions for systemic sclerosis, SLE, dermatomyositis, RA, vasculitis and APS.     

Neonatal lupus erythematosus exacerbated by vaccination

Neonatal lupus erythematosus (LE) is a rare immune-mediated disease caused by placental transport of maternal anti-SSA/Ro, anti-SSB/La and/or anti-U1RNP antibodies. Here, we demonstrate two cases of neonatal LE, in both of which cutaneous LE was exacerbated by inoculation. To our knowledge, cases worsening neonatal LE after administration of vaccines have not been reported. In case 1, not only exacerbation of pre-existing annular erythema but also spreading of new erythematous lesions to the trunk and extremities were induced following vaccination. Of interest, all of the lesions simultaneously improved. By contrast, in case 2, pre-existing facial erythema became prominent without spreading to other sites. The mother of case 1 had Sjögren’s syndrome, whereas in case 2, the mother was diagnosed with Sjögren’s syndrome on this occasion for the first time. Immunohistochemistry in case 1 revealed interleukin (IL)-17-positive cells infiltrating into the papillary dermis, and CD123-positive plasmacytoid dendritic cells in the papillary dermis and the deep reticular dermis. Both innate immune response and IL-17 mediated inflammation following vaccination are speculated as a possible mechanism of the deterioration of LE lesions in our juvenile cases. Caution is necessary since neonatal LE can be worsened following vaccination.     

2018年系统性红斑狼疮研究新进展

【摘要】 系统性红斑狼疮是一种主要累及中青年女性的复杂自身免疫疾病，临床表现多样，发病机制复杂。对于系统性红斑狼疮的研究一直是国内外自身免疫疾病领域的热点，本文主要综述2018年系统性红斑狼疮的基因组学与临床研究取得的新成果和进展。     

Atenolol oral en el manejo del hemangioma infantil: serie de casos de 46 pacientes

Propranolol, a non-selective beta-blocker, remains the first line of treatment for problematic infantile hemangioma. However, although rarely, a subset of patients experience undesirable side effects, raising interest in other selective beta-blockers. We present a large case series of 46 infants treated successfully with oral atenolol, a selective beta-1 blocker.     

Xantogranuloma juvenil: una entidad con amplio espectro clínico

Juvenile xanthogranulomas (JXGs) are rare, benign lesions that belong to the large group of non-Langerhans cell histiocytoses. JXG presents with 1 or more erythematous or yellowish nodules that are usually located on the head or neck. Most JXG lesions are congenital or appear during the first year of life. Extracutaneous involvement is rare, but the literature traditionally suggests investigating the possibility of ocular compromise. JXG is mainly a clinical diagnosis, but a skin biopsy may sometimes be needed for confirmation. JXGs on the skin are self-limiting and usually do not require treatment. This review describes the clinical and therapeutic aspects of JXG, emphasizing available evidence and the diagnosis of extracutaneous involvement.     

The Role of Multisystem Disease in Composition of Autologous Serum tears and ocular surface symptom improvement

PurposeAutologous serum tears (AST) contain growth factors and vitamins similar to those in healthy tears and are an effective treatment option for ocular surface disease. This study determined the differences in composition of AST in patients with systemic diseases versus patients with localized ocular surface diseases and the effects on ocular surface symptom improvement.MethodAn observational study was performed on 53 patients with either systemic diseases (Group I) or localized ocular surface diseases (Group II) who were prescribed AST. Concentrations of epidermal growth factor (EGF), interleukin 8 (IL-8), fibronectin, vitamin A, and tumor necrosis factor-α (TNF-α) were determined through ELISA assays from patients in both groups. The Ocular Surface Disease Index (OSDI) scores were calculated prior to and 6 weeks after initiation of treatment with AST for new patients.ResultsThe average concentration of EGF in Group I (29.39 pg/ml ± 52.85 pg/ml) was significantly lower than in Group II (88.04 pg/ml ±113.75 pg/ml) (p < 0.05). Levels of fibronectin, IL-8, and vitamin A were similar in both groups. There was a 24% reduction in OSDI score 6 weeks after initiation in Group I compared to a 36% reduction reported in Group II (p = 0.065). The OSDI score was reduced significantly after the treatment in all subjects (p = 0.002).ConclusionSerum tears are a promising therapy for management of ocular surface disease and associated symptoms. The differences between levels of EGF in patients with localized ocular surface disease and systemic inflammatory disease may account for differences in therapeutic outcome.