Indian Dermatology Online Journal最新文献

Background: Rituximab infusion and dexamethasone-cyclophosphamide pulse (DCP) are the two most popular regimens used in pemphigus vulgaris (PV) in India.

Objective: The present study compared the clinical efficacy of rituximab and DCP in Indian PV patients and their effects on serum Th1,2, and 17 cytokine levels.

Materials and methods: A total of 37 patients received DCP (Group A, n = 22) or rituximab (Group B, rheumatoid arthritis protocol (n = 15)) as per patients' preference. They were monitored for clinical response, adverse events (AEs), changes in serum anti-desmoglein-1,3 antibody titers and Th1,2 and 17 cytokine levels at baseline and weeks 20 and 52.

Results: The proportion of patients attaining disease control, remission, and relapse in groups A and B were 82% and 93%; 73% and 93%; and 27% and 50%, respectively, after a median duration of 2 months each for disease control; 4 and 4.5 months for remission; and 5 and 7 months for relapse post remission. The musculoskeletal AEs were the highest in the two groups. Significant and comparable decreases in anti-dsg1 and 3 titers from baseline to weeks 20 and 52 were observed in both groups. Th1 and Th17 cytokine levels decreased, while Th2 cytokines increased post-treatment in both groups. However, no correlation was found between change in body surface area of involvement by PV and anti-dsg titers and cytokine levels before and after therapy in both groups.

Conclusion: Comparable clinical efficacy between DCP and rituximab was observed.

International Society for the Study of Vascular Anomalies classification defines Congenital Limb Overgrowth Vascular Syndromes (CLOS) as a subset of vascular syndromes with other abnormalities that present with unilateral limb overgrowth. It includes Klippel-Trenaunay Syndrome, Parkes-Weber Syndrome, CLOVES (Congenital Lipomatous Overgrowth, Vascular Malformations, Epidermal Nevi, Spinal/Skeletal Anomalies/Scoliosis) Syndrome, Proteus Syndrome, PTEN Hamartomatous Syndrome, and Fibroadipose Vascular Anomaly. Due to their rare and complex nature, a multidisciplinary approach to diagnosis and treatment is required. A thorough clinical and radiological workup can go miles in reflecting on the patient's outcome. Here we report five cases of CLOS with their detailed dermato-radiological profiles.

[This corrects the article on p. 213 in vol. 15, PMID: 38550801.].

RASopathies refers to the group of disorders which are caused by a mutation in various genes of the RAS/MAPK (RAT sarcoma virus/Mitogen activated protein kinase) pathway. It includes many genes with varied functions, which are responsible for cell cycle regulation. As the mutation in one gene affects the entire pathway, there are many overlapping features among the various syndromes which are included under an umbrella term "RASopathies." However, neuroectodermal involvement is a unifying feature among these syndromes, which are caused by germline mutations affecting genes along this pathway. Recently, many other RASopathies have been described to involve blood vessels, lymphatics, and immune system. Also, many cutaneous mosaic disorders have been found to have mutations in the concerned pathway. The purpose of this article is to briefly review the pathogenesis of RASopathies with cutaneous manifestations, and summarise the features that can be helpful as diagnostic clues to dermatologists. As we understand more about the pathogenesis of the pathway at the cellular level, the research on genotype-phenotype correlation and therapeutic options broadens. Targeted therapy is in the clinical and preclinical trial phase, which may brighten the future of many patients.

Background: Chronic spontaneous urticaria (CSU) is a dermatosis with a significant psychological component. Only a few studies have assessed the quality of life (QoL) and perceived stress in CSU patients.

Objectives: To evaluate the QoL and perceived stress in patients with CSU.

Materials and methods: A cross-sectional study was done on 54 patients of CSU attending the Urticaria clinic of the Dermatology department in a tertiary care postgraduate teaching hospital in North Kerala. QoL in chronic urticaria was assessed using the Chronic Urticaria-Quality of Life (CU-QoL) questionnaire and perceived stress was assessed using the Perceived Stress Scale (PSS).

Results: The mean scores of CU-QoL and PSS were 55.78 and 16.31, respectively. Out of 54 patients, 26 (48.1%) had mild impairment, 26 (48.1%) had moderate impairment, and 2 (3.7%) had severe impairment of QoL. Low stress was seen in 20 (37%) patients, moderate stress in 28 (51.9%), and six (11.1%) patients had high stress.

Conclusion: This study shows that more than half of the patients with CSU had moderate to severe impairment of QoL and had moderate to severe stress.