Severe cutaneous adverse reactions: A 5-year retrospective study at Hospital Melaka, Malaysia, from December 2014 to February 2020.

INTRODUCTION: Severe cutaneous adverse reactions (SCARs) are potentially lethal adverse drug reactions that involve the skin, mucous membranes, and internal organs, resulting in disability. SCARs include drug-induced epidermal necrolysis, which is Steven Johnson syndrome (SJS)/ Steven Johnson syndrome and toxic epidermal necrolysis overlap (SJS-TEN overlap)/ toxic epidermal necrolysis (TEN), drug reaction with eosinophilia and systemic symptoms (DRESS), acute generalised exanthematous pustulosis (AGEP), generalised bullous fixed drug eruption (GBFDE), and acute erythroderma. Awareness of local epidemiology of SCARs plays an important role in prescribing practices by healthcare provider. Recognition of SCARs enables the offending drug to be withdrawn immediately, which is the definitive treatment of SCARs. MATERIALS AND METHODS: This is a retrospective study reviewing SCAR cases reported to the Malaysian Adverse Drug Reactions Advisory Committee (MADRAC) registry at the Department of Dermatology, Hospital Melaka, for 5 years and 3 months from December 2014 to February 2020. RESULTS: A total of 41 SCARs cases were identified over the study duration. The incidence rate was 0.18%. All 41 cases require hospitalisations, with four cases (9.8%) managed in ICU and one mortality (2.4%) due to SJS-related complication. One patient had two episodes of SCARs. There were 22 male patients and 18 female patients. The majority were Malays (33, 80.5%), followed by Chinese (7, 17.1%) and Indonesian (1, 2.4%). There was no Indian patient with SCARs in this study. The mean age of patients was 47.2±17 years. Drug-induced epidermal necrolysis was the commonest type of SCARs (63.4%), and out of this, SJS accounted for the majority of cases (48.8%). Antibiotic was the main group of offending medication in this SCAR study (29.3%). The top five individual causative drugs of SCARs in sequence include allopurinol, phenytoin, carbamazepine, co-amoxiclav, and cephalexin. Allopurinol was the commonest culprit drug for drug-induced epidermal necrolysis and DRESS, phenytoin for acute erythroderma, and co-amoxiclav for AGEP. CONCLUSION: SJS was the most common manifestation and Allopurinol was the commonest culprit drug for SCAR cases in our cohort.

Characteristics and quality of life in pemphigus patients.

INTRODUCTION: Pemphigus is an autoimmune blistering disease affecting the skin and mucus membranes. It is a debilitating skin condition with painful bullae and erosions, which may limit the patient's daily activities. Therefore, measuring the quality of life (QoL) from the perspective of physical, functional, social, and emotional well-being is important to address the disease burden. This study aims to review the demography and assess the impact of disease on QoL in pemphigus patients at the Department of Dermatology, Hospital Melaka. MATERIALS AND METHODS: This is a single-centre, crosssectional study on the characteristics and QoL among the pemphigus patients at the Department of Dermatology, Melaka General Hospital, from August 2020 to July 2021. Patients' information was collected, and each patient was assessed objectively on the disease severity physically using the Pemphigus Disease Area Index (PDAI) scoring system. The disease severity was then assessed subjectively, in which each participant was given three questionnaires to answer, namely the Dermatology Life Quality Index (DLQI), Visual Analogue Scale (VAS) for pain and itch, and Autoimmune Bullous Disease Quality of Life (ABQOL). RESULTS: There were a total of 30 pemphigus patients (13 males, 17 females), with an average age of 54.0 ± 13.6 years. Our study population had low median PDAI score (2.0 ± 4.0) with low median DLQI (3.0 ± 8.0) and ABQOL (11.0 ± 12.0). The median VAS scores for pain (1.0 ± 2.0) and itch (2.0 ± 3.0) were also low. Patients with tertiary educational qualification reported higher median DLQI (10.0 ± 12.0, p = 0.016) and ABQOL (21.0 ± 23.0, p = 0.026). Significant correlation was neither observed between PDAI and DLQI scores nor observed between PDAI and ABQOL scores. The DLQI and ABQOL scores were not affected by gender, age, ethnicity, and duration of illness. CONCLUSION: Most of the patients in our study cohort had low DLQI and ABQOL scores, with mild clinical severity, as evidenced by low PDAI and VAS scores for both pain and itch. Disease severity had no correlation with QoL in our study. However, educational level showed significant influence on the QoL.

Skin- and gut-homing molecules on human circulating γδ T cells and their dysregulation in inflammatory bowel disease.

Changes in phenotype and function of γδ T cells have been reported in inflammatory bowel disease (IBD), including Crohn's disease (CD) and ulcerative colitis (UC). Dysregulation of lymphocyte migration plays a key role in IBD pathogenesis; however, data on migratory properties of γδ T cells are scarce. Human circulating γδ T cells from healthy controls (n = 27), patients with active CD (n = 15), active UC (n = 14) or cutaneous manifestations of IBD (n = 2) were characterized by flow cytometry. Circulating γδ T cells in healthy controls were CD3(hi) and expressed CD45RO. They expressed gut-homing molecule ß7 but not gut-homing molecule corresponding chemokine receptors (CCR)9, or skin-homing molecules cutaneous lymphocyte-associated antigen (CLA) and CCR4, despite conventional T cells containing populations expressing these molecules. CCR9 expression was increased on γδ T cells in CD and UC, while skin-homing CLA was expressed aberrantly on γδ T cells in patients with cutaneous manifestations of IBD. Lower levels of CD3 expression were found on γδ T cells in CD but not in UC, and a lower proportion of γδ T cells expressed CD45RO in CD and UC. Enhanced expression of gut-homing molecules on circulating γδ T cells in IBD and skin-homing molecules in cutaneous manifestations of IBD may be of clinical relevance.