Acral Folliculocentric Extragenital Lichen Sclerosus: A Case Report.

Introduction: Lichen sclerosus is a chronic inflammatory dermatological condition of unknown etiology, primarily impacting the genital epidermis in individuals of all genders, with a higher prevalence observed among postmenopausal women and prepubescent girls. Additionally, extragenital manifestations occur in approximately 20% of the patients diagnosed with genital lichen sclerosus. Notably, folliculocentric extragenital lichen sclerosus is rare and unusual, with only limited instances documented in existing literature. Case Description: We report a 33 years old lady presented with multiple asymptomatic lesions on the dorsal feet for 1 year and similar lesions on the left hand for 4 months. On examination: folliculocentric, shiny, atrophic papules coalescing into reticulated plaques over the dorsum of both feet and few shiny, flat-topped, pink papules over the dorsum of the left hand. A skin biopsy was performed and confirmed the diagnosis of extragenital lichen sclerosus. Conclusion: Acral folliculocentric extragenital lichen sclerosus is an unusual and rare clinical variant. Clinicopathologic correlation is necessary to establish the correct diagnosis. Contribution to the Literature: Herein, we present an unusual presentation of extragenital lichen sclerosus, and we highlight the importance of considering it in the differential diagnosis of guttate acral skin lesions. We also review and summarize relevant cases from the literature in hope to aid physicians, especially dermatologists, to consider and swiftly reach the diagnosis and offer appropriate management. We also hope to bring about new insights and broaden future research efforts regarding lichen sclerosus especially and atrophic skin disease in general.

Delayed Inflammatory Reaction to Hyaluronic Acid Dermal Filler Following Zoledronic Acid Administration: A Case Report.

Zoledronic acid is a bisphosphonate that can be administered intravenously and used to treat several bone disorders. It decreases bone resorption, thereby improving bone mineral density (BMD) and reducing fractures. The Food and Drug Administration (FDA) has approved zoledronic acid for the prevention and treatment of osteoporosis in postmenopausal females and males and for other conditions. Zoledronic acid is generally well tolerated, with most side effects being musculoskeletal or gastrointestinal. Cutaneous side effects include maculopapular rash and other mild skin reactions. Rare severe skin rashes, such as toxic epidermal necrolysis, have been reported. Here, we report the case of a 64-year-old female with a medical history of breast cancer status post-radical mastectomy and chemotherapy presenting with delayed hypersensitivity reaction to a hyaluronic acid dermal filler two days after receiving zoledronic acid intravenously given to maintain bone density, symptoms completely resolved with oral prednisolone 20 mg once daily and cetirizine 10 mg. Cases of delayed inflammatory reaction to hyaluronic acid soft tissue filler have previously been reported in patients who have received vaccination or those with viral infections. However, to our knowledge, there have been no reports of delayed inflammatory reactions to facial hyaluronic acid injections after zoledronic acid administration.

Synthetic Hybrid-Scale Fiber Matrix for the Treatment of Complex Lower-Extremity Wounds.

BACKGROUND: Chronic, nonhealing wounds are a growing health-care problem in the United States, affecting more than 6.5 million patients annually and costing the health-care system over $25 billion. Chronic wounds, including diabetic foot ulcers (DFUs) and venous leg ulcers (VLUs), are often difficult to treat, and patients commonly fail to heal even with the most advanced therapies. The present study was designed to evaluate the efficacy and utility of the synthetic hybrid-scale fiber matrix in the treatment of complex chronic nonhealing lower-extremity ulcers refractory to advanced therapies. METHODS: A retrospective analysis of 20 patients with a total of 23 wounds (DFUs, n = 18; VLUs, n = 5) who underwent treatment using the synthetic hybrid-scale fiber matrix was conducted. The majority of ulcers (78%) included in this study were refractory to one or multiple previous advanced wound therapies and therefore considered difficult-to-heal ulcers with high failure risk for future therapies. RESULTS: Subjects had a mean wound age of 16 months and presented with 132 secondary comorbidities and 65 failed interventions and therapies. Treatment of VLUs with the synthetic matrix resulted in complete closure of 100% of the wounds over 244 ± 153 days with an average of 10.8 ± 5.5 applications. Treatment of DFUs with the synthetic matrix resulted in complete closure of 94% of the wounds over 122 ± 69 days with 6.7 ± 3.9 applications. CONCLUSIONS: Treatment with the synthetic hybrid-scale fiber matrix resulted in the closure of 96% of complex chronic ulcers refractory to existing therapies. The inclusion of the synthetic hybrid-scale fiber matrix in wound care programs provides a critical and needed solution for costly, long-standing refractory wounds.

Awareness of food allergies: a survey of pediatricians in Kuwait.

BACKGROUND: Early diagnosis of food allergies (FA) is important for a favorable prognosis. This study aimed to determine the level of awareness of FA among pediatricians in Kuwait. METHOD: A 43-item self-administered questionnaire was designed and distributed to pediatricians working at 4 government hospitals in Kuwait. RESULTS: A total of 140 pediatricians completed the questionnaire, with a participation rate of 51.1% (81 males and 59 females). The mean age of participants was 40.81 years, and the mean number of years working in pediatrics was 13.94 years. The mean overall knowledge score was 22.2. The pediatricians' overall knowledge scores were found to be significantly associated with their age (older pediatricians had higher overall scores) and years of experience as a pediatrician but were independent from hospital site, gender, or rank. A multiple linear regression revealed pediatrician age and gender were the only variables that were significantly associated with the overall knowledge score. Only 16.4% of the participants answered at least 2/3 of the survey questions correctly. The questions that were correctly answered by ≤ 2/3 of the participants constituted 80% of clinical presentation questions, 66.6% of diagnostics questions, 77.7% of treatment questions, and 42.8% of prevention questions. Interestingly, among 68 pediatricians (48.5%) who determined that they felt comfortable evaluating and treating patients with FA, only 12 (17.6%) passed the questionnaire. CONCLUSIONS: This survey demonstrates that there is a noteworthy deficiency of pediatricians' awareness about FA. The implementation of strategies to improve pediatricians' awareness is critical to diagnose food allergy patients early and improve their health and outcomes.

Loss of interleukin-10 signaling and infantile inflammatory bowel disease: implications for diagnosis and therapy.

BACKGROUND & AIMS: Homozygous loss of function mutations in interleukin-10 (IL10) and interleukin-10 receptors (IL10R) cause severe infantile (very early onset) inflammatory bowel disease (IBD). Allogeneic hematopoietic stem cell transplantation (HSCT) was reported to induce sustained remission in 1 patient with IL-10R deficiency. We investigated heterogeneity among patients with very early onset IBD, its mechanisms, and the use of allogeneic HSCT to treat this disorder. METHODS: We analyzed 66 patients with early onset IBD (younger than 5 years of age) for mutations in the genes encoding IL-10, IL-10R1, and IL-10R2. IL-10R deficiency was confirmed by functional assays on patients' peripheral blood mononuclear cells (immunoblot and enzyme-linked immunosorbent assay analyses). We assessed the therapeutic effects of standardized allogeneic HSCT. RESULTS: Using a candidate gene sequencing approach, we identified 16 patients with IL-10 or IL-10R deficiency: 3 patients had mutations in IL-10, 5 had mutations in IL-10R1, and 8 had mutations in IL-10R2. Refractory colitis became manifest in all patients within the first 3 months of life and was associated with perianal disease (16 of 16 patients). Extraintestinal symptoms included folliculitis (11 of 16) and arthritis (4 of 16). Allogeneic HSCT was performed in 5 patients and induced sustained clinical remission with a median follow-up time of 2 years. In vitro experiments confirmed reconstitution of IL-10R-mediated signaling in all patients who received the transplant. CONCLUSIONS: We identified loss of function mutations in IL-10 and IL-10R in patients with very early onset IBD. These findings indicate that infantile IBD patients with perianal disease should be screened for IL-10 and IL-10R deficiency and that allogeneic HSCT can induce remission in those with IL-10R deficiency.

Complications in paediatric liver transplant from Kuwait when transplanted abroad.

BACKGROUND AND STUDY AIMS: Since the introduction of liver transplantation (LTx) in children suffering from liver failure in 1963, many centres around the world have offered this service to children that have no other alternative. The aim of this retrospective study is to analyse the results of paediatric liver transplant in Kuwait over the last decade. PATIENTS AND METHODS: A retrospective chart review was done involving paediatric patients during the time period of 1995-2004. The information collected included patient demographics, indications for liver transplantation, survival of both patient and allograft, and complications. RESULTS: A total of 16 cases were found and analysed. The mean age was 3.6years (ranged 5months-17years). There were nine boys and seven girls. The most common indications for LTx were biliary atresia and metabolic liver disease. All the liver transplants were done abroad. There were totally nine deceased donor and seven living related cases. The complications were acute cellular rejection in five, hypertension in two, biliary complications in four, cytomegalovirus (CMV) infection in three and post lymphoproliferative disease in two cases. All but one patient are presently alive. CONCLUSION: The above information demonstrates that LTx in Kuwati children is safe and improves the quality of life for those that would otherwise have no other alternative.

Performance status and deaths among children registered in Kuwait National Primary ImmunoDeficiency Disorders Registry.

Children with primary immunodeficiency disorders (PIDD) have an increased risk of suffering from physical, social, and psychological problems. The aim of this study was to evaluate the performance status and mortality of children with PIDD in Kuwait and to determine the variables and co-morbidities that may affect their performance and risk of death. The data for the children were obtained from Kuwait National Primary Immunodeficiency Disorders Registry describes the patients' characteristics, comorbidities and their treatment regimens. Each patient was scored using the Lansky Play Performance Scale (LPPS), and we evaluated the number of deaths among the children and the effects of different variables on their LPPS scores and mortality. We examined 98 pediatric patients with a mean delay in diagnosis of 21.2 months. Antimicrobial prophylaxis was administered to 57.2% of the patients, whereas intravenous immunoglobulin (IVIG) therapy was used in 44%. Eight patients underwent bone marrow transplants. The mean LPPS score for all the patients was 65.5, and there was a significant disparity in the mean LPPS scores across PIDD categories. Twenty-one patients died. The variables that were found to have a significant effect on both the LPPS score and the risk of death were an age of onset of less than 6 months, a history of CMV infection, parental consanguinity, the use of antimicrobial prophylaxis and IVIG therapy. In conclusion, patients with PIDD have a poor performance status and a high rate of mortality. Early diagnosis and aggressive therapeutic interventions directed at patients with early onset of symptoms and CMV infections can help improve the quality of life of patients with PIDD.

Primary immunodeficiency disorders: survey of pediatricians in Kuwait.

INTRODUCTION: Early diagnosis of primary immunodeficiency disorders (PID) is critical so life saving interventions can be implemented to avoid significant morbidity and mortality. Unfortunately, they are frequently misdiagnosed, which results into significant delay in diagnosis. This study aimed to determine the knowledge and practice of pediatricians in Kuwait about PID. MATERIALS AND METHODS: A 66-item self-administered questionnaire was designed and distributed to the pediatricians working at all six governmental hospitals to measure their knowledge and practice about PID. A total of 244 pediatricians (78.4%; 143 males and 101 females) participated in the study. The mean age of participants was 40 years, and the mean number of years working in pediatrics was 13 years. The mean overall score was 59.6%, whereas the mean score in clinical presentation section was 63%, in associated diseases and syndromes section 58%, and in laboratory investigations section 51%. Only 26% of the participants answered correctly at least 2/3 of the questions (67% of the questions). CONCLUSION: This survey demonstrates that there is universal deficiency in both the knowledge and practice of pediatricians in the field of PID. Implementation of strategies to improve the awareness of pediatricians about PID is critical so early therapeutic interventions can be done to improve the health and prevent morbidity and mortality.

Epidemiology and outcome of severe hepatitis A infection in children in Kuwait.

OBJECTIVE: To describe the epidemiology, clinical features and outcome of hepatitis A virus (HAV) infection in children in Kuwait. SUBJECTS AND METHOD: The medical records of 350 patients (age 0-16 years) admitted to the Infectious Diseases Hospital, Kuwait, between January 2000 and December 2002, with hepatitis A infection were reviewed. RESULTS: The mean age was 8.6 +/- 3.8 years and 47% of patients were between 7 and 12 years old. Kuwaiti children comprised 44% of admitted patients. The largest proportion of children with hepatitis A (34%) were from the northern part of the country. HAV infection was prevalent throughout the year with a peak during the months of August to October. Of the Kuwaiti children, 31% reported a history of contact with jaundiced patients, while 52% of the non-Kuwaiti children had a history of recent travel prior to their illness. Patients had symptoms for a mean of 6 +/- 3.6 days prior to presentation. The mean length of hospitalization was 5.8 +/- 2.9 days. The rate of complications of HAV infection was 6% and only one child required admission to the intensive care unit for fulminant hepatitis. None of the patients had permanent sequelae. CONCLUSIONS: HAV infection is a significant cause of morbidity for children in Kuwait. The disease is mostly prevalent in preschool and school age children. Despite the excellent outcome of all patients, a considerable number of patients tend to have a complicated course and prolonged hospitalization. In view of these data, hepatitis A vaccine should be considered as a part of routine childhood immunization in Kuwait.