ABSTRACT
Common autoimmune bullous diseases (AIBDs) include pemphigus and bullous pemphigoid (BP), which are primarily caused by IgG autoantibodies against the structural proteins of desmosomes at the cell-cell junction and hemidesmosomes at the epidermal-dermal junction. Few studies have assessed nail changes in patients with pemphigus or BP. In the present study, we collected the clinical data of 191 patients with AIBDs (108 patients with pemphigus and 83 patients with BP) and 200 control subjects. Nail changes were observed in 77.0% (147/191), 77.8% (84/108), and 75.9% (63/83) of patients with AIBDs, pemphigus, and BP, respectively, and 14.5% (29/200) of control subjects. Beau's lines and paronychia were the most common nail involvement, observed in 22.5% (43/191) and 22.5% (43/191) of patients with AIBDs, 25.0% (27/108) and 25.9% (28/108) of patients with pemphigus, 19.3% (16/83) and 18.1% (15/83) of patients with BP, respectively. The autoimmune bullous skin disorder intensity score (ABSIS) and the onset time of patients with pemphigus or BP with nail changes were different. Onychomycosis accounted for 21.5% (41/191) of all patients with AIBDs. The ABSIS was correlated with nail involvement in patients with BP (r = 0.46, p < 0.001), and weakly correlated with nail involvement in patients with AIBDs (r = 0.37, p < 0.001), pemphigus (r = 0.29, p = 0.009), and pemphigus vulgaris (PV; r = 0.35, p = 0.008). No correlation was observed between nail involvement and disease antibody titers. In conclusion, nail changes are frequently observed in patients with pemphigus and BP. The type and onset time of nail changes may indicate the severity of pemphigus and BP, which warrants the attention of dermatologists.
ABSTRACT
PURPOSE: Acute poisoning is a major cause of disability and death in children, but there is a lack of large-scale and multicenter epidemiological data on acute poisoning in children. PATIENTS AND METHODS: The current study was a retrospective analysis of clinical data from 1755 children aged 0 to 14 years who were hospitalized between 01 January 2014 and 31 December 2020 in southwestern China. RESULTS: Acute poisoning was common in childhood, particularly in early childhood (607; 34.6%) and preschool (655; 37.3%), and most cases occurred in rural areas (1191; 67.9%). It was more common in boys (934; 53.2%) than in girls (821; 46.8%). In urban areas, poisoning was mainly caused by drugs (266; 47.2%), chemical agents (59; 10.5%), and alcohol (54; 9.6%). In rural areas, it was mainly caused by pesticides (620; 52.1%) and medications (213; 17.9%), the route of poisoning was usually the digestive tract (1671; 95.2%), in most cases the poisoning was accidental (1618; 92.3%), and pesticides (659; 37.5%) and medications (479; 27.3%) predominated. After timely treatment, the majority of patients had no substantial organ damage, sequelae, or disabilities, and mortality was low (17; 1.0%). CONCLUSION: Acute poisoning is a frequent accident in childhood, mainly in infants and preschool children, mostly in rural areas, and poisoning mostly occurs via the digestive tract. Family and community education should be conducted to prevent poisoning.
ABSTRACT
We report a case of a 3-year-old girl with generalized pustular psoriasis (GPP) and a compound heterozygous mutation within the IL36 receptor antagonist (IL36RN) gene at c.28C>T and c.115+6T>C, who was successfully treated using secukinumab monotherapy. The first dose of secukinumab (150 mg) was administered subcutaneously, followed by 75 mg of secukinumab subcutaneously once weekly for 4 weeks and then 75 mg every 4 weeks. The total course of treatment was 4 months, with a total of 8 doses. A PASI score of 75 and 100 was achieved at weeks 1 and 2, respectively, and maintained for 11 months of follow-up, without disease recurrence or adverse events.
Subject(s)
Interleukins , Psoriasis , Antibodies, Monoclonal, Humanized , Child, Preschool , Female , Humans , Interleukins/genetics , Mutation , Psoriasis/drug therapy , Treatment OutcomeABSTRACT
A 22-year-old primigravida had a pruritic, erythematous, bullous eruption on the skin during the 26th week of gestation. After delivery the eruption flared up. The diagnosis of pemphigoid gestationis was confirmed based on histopathological and immunofluorescence findings. The result of immunoblotting showed IgG autoantibodies which reacted against BP230 in epidermal extracts and 290 kDa type VII collagen in dermal extracts. The BP180 antibodies were also detected by an enzyme-linked immunosorbent assay BP180NC16a diagnosis kit. Pulsed corticosteroid and cyclophosphamide resulted in a favourable response at the acute stage. The patient was cured in 2 years. The analysis of the patient's autoantibodies provides strong evidence for the involvement of epitope spreading in her autoimmune disease.
