Background
Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe adverse drug reaction including integumentary and internal organs. An extensive literature review of DRESS in the pediatric population has not been performed.
Methods
A literature search was performed to find reports of pediatric DRESS published between 1997 and March 2019. If not already included, each case was scored based on RegiSCAR criteria. Only “probable” or “definite” cases of DRESS were included in the analysis, totaling 130 cases.
Results
In the pediatric population, the average age of diagnosis for DRESS was 8.7 years old. The most common causative drugs include antiepileptics (50%) and antibiotics (30.8%). Time from drug exposure to DRESS presentation averaged 23.8 days. Common clinical symptoms include rash (99.2%) (typically morbilliform (89.2%)), fever (96.2%), eosinophilia (90%), and lymphadenopathy (74.6%). Human herpesvirus‐6 reactivation was observed in 16.1% of cases. The most commonly affected internal organ was the liver (80%), followed by the spleen (21.5%) and kidney (15.4%). Management strategies involved, either alone or in combination, included corticosteroids (intravenous 60.8% and oral 41.5%), intravenous immunoglobulins (12.3%), plasmapheresis (2.3%), and ganciclovir (1.5%). Long‐term sequelae occurred in 10.8% of patients, most commonly hypothyroidism (3.8%), liver failure (3.1%), and diabetes (2.3%). The mortality rate was 5.4%.
Conclusion
This literature review highlights the presentation and course of pediatric DRESS. Morbilliform eruption, fever, and eosinophilia appear to be clinical hallmarks of pediatric DRESS. Common causative agents, specifically carbamazepine, are comparable to the adult population. Furthermore, the mortality rate from DRESS is significant and is similar between pediatric and adult patients.
Atopic dermatitis (AD) is a chronic inflammatory skin condition that can have tremendous impact on quality of life for affected children and adults. First-line therapy for acute management of AD includes topical therapies such as corticosteroids, calcineurin inhibitors, and, more recently, the phosphodiesterase inhibitor crisaborole. Topical agents have remained the mainstay therapy for decades; however, there has been a longstanding need for topical therapies with high efficacy and low risk of adverse effects with long-term use. Given the ongoing advances in understanding the pathogenesis of AD, there are novel targets for pharmacological intervention. We are now in an unprecedented time with more than 40 topical treatments in the pipeline for AD in addition to many developments and treatments on the horizon. This review summarizes selected therapeutic topical agents in later phases of development that target various aspects in the pathogenesis of AD such as Janus kinase inhibition (ruxolitinib and delgocitinib), phosphodiesterase-4 inhibition (roflumilast and difamilast), aryl hydrocarbon modulation (tapinarof), and modulation of the microbiome. We also review novel targeted therapies that are in early phase clinical trials, including AMTX-100, BEN-2293, and PRN473. Preliminary findings on efficacy and tolerability of most of these agents are promising, but further studies are warranted to evaluate the long-term safety and efficacy of these novel agents against the current standard of care.
Background
There are no widely accepted pediatric guidelines on who should undergo biopsy of the nail apparatus to screen for subungual melanoma (SUM). Reported cases of pre-adolescent children diagnosed with SUM presenting as melanonychia remain controversial. Further, observed age differences in the incidence of acral lentiginous melanoma are complicated by reported ethnic differences in adult prevalence and survival rates.
Methods
We conducted a retrospective chart review of Rady Children's Hospital San Diego, a tertiary hospital system, with over 2 million patients in its electronic health records to identify patients diagnosed with melanonychia younger than 12 years and biopsies in these children, between January 1, 2010, and July 31, 2021.
Results
There were 623,805 patients younger than 12 years of age seen in the outpatient setting. Average age was 7.2 years for melanonychia diagnosis, and 5.1 years for those biopsied. Nail apparatus biopsies were performed in 22 different individuals and involved the hand 17/22 (77%) and the foot 5/22 (23%). The presence of Hutchinson's sign was documented in 9/22 (41%) patients. Males were diagnosed with melanonychia and underwent biopsy more often than females. Many of the biopsies were performed in Hispanic/Latino 11/22 (50%) and Asian/Pacific Islander 3/22 (14%) patients.
Discussion
Regardless of reported ethnicity, biopsy of nail apparatus is highly unlikely to uncover invasive ALM in children younger than 12 years. Prospective studies are needed to understand how incidence of melanonychia, age, gender, and ethnicity/race influence parental and clinicians' perceptions of melanoma risk.
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