Divergent clinical presentations and outcomes among children and adolescents with Kaposi sarcoma in Malawi and Tanzania.

OBJECTIVES: The Kaposi sarcoma (KS) T0 versus T1 staging classification does not address the unique clinical features of paediatric KS in human gammaherpesvirus 8 (HHV-8) endemic regions of Africa. This study seeks to define patterns of childhood KS using a paediatric-specific approach. METHODS: The Lilongwe paediatric KS staging classification categorizes disease based on clinical phenotype: stage 1 = mild/moderate KS limited to cutaneous/oral involvement, stage 2 = primarily lymphadenopathic disease, stage 3 = woody edema KS, stage 4 = visceral and/or severe/disseminated mucocutaneous disease. Characteristics and outcomes were evaluated from paediatric referral centres in Lilongwe, Malawi, and Mbeya, Tanzania. RESULTS: Among 171 patients, the median age was 9.3 years, 37% (n = 63) were female, and 87% (n = 149) had HIV. Breakdown by stage was as follows: 18% (n = 31) stage 1, 33% (n = 56) stage 2, 19% (n = 33) stage 3, and 30% (n = 51) stage 4. Age (younger stage 2 and older stage 3), severe CD4 count suppression (lower CD4 for stages 1 and 4), and presence of severe anaemia and thrombocytopenia (worse for stages 2 and 4) differed across stages. Estimated 2-year event-free survival/progression-free survival/overall survival by stage was as follows: stage 1, 81%/81%/87%; stage 2, 50%/50%/63%; stage 3, 24%/49%/81%; and stage 4, 29%/34%/54%. Sub-analysis of stage 2 lymphadenopathic KS demonstrated superior long-term 6-year event-free survival of 70% (95% confidence interval [CI] 49-83) for younger children (aged <7 years) versus 27% (95% CI 8-51) for older children. CONCLUSIONS: This paediatric-specific staging classification categorizes patients with distinct characteristics and patterns of treatment response. This platform may guide clinicians to provide risk-stratified treatment with the hope of improving survival among children with KS.

Long-term outcomes for children and adolescents with Kaposi sarcoma.

OBJECTIVES: Kaposi sarcoma (KS) is one of the most common childhood cancers in eastern and central Africa. It has become a treatable disease with increasing availability of antiretroviral therapy (ART) and chemotherapy. We aimed to fill the data gap in establishing whether long-term survival is achievable for children in low-income countries. METHODS: We retrospectively analysed data for children and adolescents aged ≤ 18.9 years diagnosed with HIV-related or endemic KS from 2006 to 2015 who received standardized institutional treatment regimens utilizing chemotherapy plus ART (if HIV-positive) at a tertiary care public hospital in Lilongwe, Malawi. Long-term survival was analysed and mortality was associated with KS for those with refractory/progressive disease at the time of death. RESULTS: There were 207 children/adolescents with KS (90.8% HIV-related); 36.7% were alive, 54.6% had died, and 8.7% had been lost to follow-up. The median follow-up time for survivors was 6.9 years (range 4.2-13.9 years). Death occurred at a median of 5.3 months after KS diagnosis (range 0.1-123 months). KS progression was associated with mortality for most (61%) early deaths (survival time of < 6 months); conversely, KS was associated with a minority (31%) of late-onset deaths (after 24 months). The 7-year overall survival was 37% [95% confidence interval (CI) 30-44%] and was higher for those diagnosed between 2011 and 2015 compared to 2006-2010: 42% (95% CI 33-51%) versus 29% (95% CI 20-39%), respectively (P = 0.01). Among the 66 HIV-positive survivors, 58% were still on first-line ART. CONCLUSIONS: Long-term survival is possible for pediatric KS in low-resource settings. Despite better survival in more recent years, there remains room for improvement.

Clinical and pathologic correlation of cutaneous COVID-19 vaccine reactions including V-REPP: A registry-based study.

BACKGROUND: Cutaneous reactions after COVID-19 vaccination have been commonly reported; however, histopathologic features and clinical correlations have not been well characterized. METHODS: We evaluated for a history of skin biopsy all reports of reactions associated with COVID-19 vaccination identified in an international registry. When histopathology reports were available, we categorized them by reaction patterns. RESULTS: Of 803 vaccine reactions reported, 58 (7%) cases had biopsy reports available for review. The most common histopathologic reaction pattern was spongiotic dermatitis, which clinically ranged from robust papules with overlying crust, to pityriasis rosea-like eruptions, to pink papules with fine scale. We propose the acronym "V-REPP" (vaccine-related eruption of papules and plaques) for this spectrum. Other clinical patterns included bullous pemphigoid-like (n = 12), dermal hypersensitivity (n = 4), herpes zoster (n = 4), lichen planus-like (n = 4), pernio (n = 3), urticarial (n = 2), neutrophilic dermatosis (n = 2), leukocytoclastic vasculitis (n = 2), morbilliform (n = 2), delayed large local reactions (n = 2), erythromelalgia (n = 1), and other (n = 5). LIMITATIONS: Cases in which histopathology was available represented a minority of registry entries. Analysis of registry data cannot measure incidence. CONCLUSION: Clinical and histopathologic correlation allowed for categorization of cutaneous reactions to the COVID-19 vaccine. We propose defining a subset of vaccine-related eruption of papules and plaques, as well as 12 other patterns, following COVID-19 vaccination.

Novel Education Modules Addressing the Underrepresentation of Skin of Color in Dermatology Training.

BACKGROUND: Representative images of pathology in patients with skin of color are lacking in most medical education resources. This particularly affects training in dermatology, which relies heavily on the use of images to teach pattern recognition. The presentation of skin pathology can vary greatly among different skin tones, and this lack of representation of dark skin phototypes challenges providers' abilities to provide quality care to patients of color.In Botswana and other countries in sub-Saharan Africa, this challenge is further compounded by limited resources and access to dermatologists. There is a need for improved and accessible educational resources to train medical students and local medical providers in basic skin lesion description and diagnosis. OBJECTIVES: We examined whether online Perceptual and Adaptive Learning Modules (PALMs) composed of representative dark skin images could efficiently train University of Botswana medical students to more accurately describe and diagnose common skin conditions in their community. METHODS: Year 4 and 5 medical students voluntarily completed PALMs that teach skin morphology, configuration, and distribution terminology and diagnosis of the most common dermatologic conditions in their community. Pre-tests, post-tests and delayed-tests assessed knowledge acquisition and retention. RESULTS: PALMs training produced statistically significant (P < .0001) improvements in accuracy and fluency with large effect sizes (1.5, 3.7) and good retention after a 12.5-21-week median delay. Limitations were a self-selected group of students, a single institution, slow internet connections, and high drop-out rates. CONCLUSIONS: Overall, population-specific PALMs are a useful tool for efficient development of pattern recognition in skin disease description and diagnosis.

Prospective Implementation of a Consultative Store-and-Forward Teledermatology Model at a Single Urban Academic Health System with Real Cost Data Subanalysis.

Background: Teledermatology may increase access to care but has not been widely implemented due, in part, to lack of insurance coverage and reimbursement. We assessed the impact of implementing a consultative store-and-forward teledermatology model on access to care, medical cost, and utilization. Materials and Methods: Prospective implementation of teledermatology occurred at five University of Pennsylvania Health System primary care practices from June 27, 2016, to May 25, 2017. Primary outcomes included time to case completion, proportion of patients completing in-person dermatology visits, and total outpatient costs. Medical and pharmacy claims data were used for utilization and cost subanalysis. Results: The study included 167 patients and 1,962 controls with a 6-month follow-up. Median time to definitive dermatologist response was 0.19 days (interquartile range [IQR]: 0.03-2.92) for intervention and 83.60 days (IQR: 19.74-159.73) for controls. In medical claims subanalysis, no significant differences in mean outpatient costs ($3,366 vs. $2,232, p = 0.1356) or total medical costs ($3,535 vs. $2,654, p = 0.2899) were detected. Conclusions: Implementation of teledermatology improved access to care, and within this small sample, remained comparable in terms of cost and utilization. Thus, these data suggest teledermatology may improve access without increasing utilization or cost.

Disseminated cysticercosis and Kaposi sarcoma in a child with HIV/AIDS: A case report.

BACKGROUND: Clinical manifestations of extraneural infection with the pork tapeworm Taenia solium typically affect the muscles, eyes, alimentary canal, and/or subcutaneous tissues. Children living with HIV are at increased risk for more widespread and severe manifestations of food-borne opportunistic infections, including T. solium, due to fluctuating levels of immunosuppression. We present a case of disseminated T. solium in a HIV-positive child with Kaposi sarcoma living in Tanzania with cysticercosis presenting as widespread subcutaneous nodules. CASE PRESENTATION: A 4-year-old HIV-positive boy in Southern Tanzania presented for evaluation of > 30 violaceous skin lesions, few subcutaneous nodules, and a circumferential violaceous penile lesion which rapidly grew after initiation of ART. The patient was clinically diagnosed with Kaposi sarcoma and started on chemotherapy with bleomycin, vincristine, and doxorubicin. He completed 10 cycles of chemotherapy, with full resolution of the violaceous skin and penile lesions but persistence of his subcutaneous nodules, thus paclitaxel was added. After 12 additional cycles of paclitaxel, his subcutaneous nodules enlarged, and biopsy of a scapular subcutaneous nodule was performed. Histopathology revealed a cystic structure with a central larval scolex and serrated spiral canal consistent with T. solium, which confirmed a diagnosis of disseminated cysticercosis. He completed a 10-day course of praziquantel and albendazole with resolution of the subcutaneous nodules. CONCLUSIONS: Disseminated cysticercosis is an unusual opportunistic infection which can present as subcutaneous nodules without other typical cysticercosis symptoms. Immunosuppression - from HIV and/or chemotherapy - may unmask cysticercosis in children in endemic regions and result in more severe manifestations of this disease. Cysticercosis should remain on a clinician's differential for subcutaneous nodules, especially in children living with HIV. Cysticercosis can mimic Kaposi sarcoma, and histopathology is essential to accurately diagnose and manage patients with concerning skin lesions.

Sexually acquired syphilis: Historical aspects, microbiology, epidemiology, and clinical manifestations.

Syphilis is caused by infection with the spirochetal bacterium Treponema pallidum subsp. pallidum. It was first recognized in the late 15th century. Since 2000, the incidence of sexually acquired syphilis has increased substantially in the developed world, with men who have sex with men and persons living with HIV infection disproportionately affected. Clinical manifestations of syphilis are protean and often include mucocutaneous manifestations. The first article in this continuing medical education series reviews historical aspects, microbiology, epidemiology, and clinical manifestations of sexually acquired syphilis.

Sexually acquired syphilis: Laboratory diagnosis, management, and prevention.

The methods used for the laboratory diagnosis of syphilis include direct detection of Treponema pallidum subspecies pallidum and serologic testing. Serologic testing relies on both nontreponemal and treponemal tests. In newly developed reverse-sequence screening algorithms, treponemal tests are performed before nontreponemal tests. The management of syphilis requires appropriate staging, treatment, and follow-up of patients along with the prompt reporting of infections to public health authorities to assist with prevention and control efforts. Benzathine penicillin G remains the treatment of choice for all stages of syphilis. Screening of populations at higher risk for syphilis is recommended by the US Centers for Disease Control and Prevention, the US Preventive Services Task Force, and the World Health Organization. The second article in this continuing medical education series reviews the testing for and the management of sexually acquired syphilis.

Clinical effectiveness and cost-effectiveness of teledermatology: Where are we now, and what are the barriers to adoption?

There has been rapid growth in teledermatology over the past decade, and teledermatology services are increasingly being used to support patient care across a variety of care settings. Teledermatology has the potential to increase access to high-quality dermatologic care while maintaining clinical efficacy and cost-effectiveness. Recent expansions in telemedicine reimbursement from the Centers for Medicare & Medicaid Services (CMS) ensure that teledermatology will play an increasingly prominent role in patient care. Therefore, it is important that dermatologists be well informed of both the promises of teledermatology and the potential practice challenges a continuously evolving mode of care delivery brings. In this article, we will review the evidence on the clinical and cost-effectiveness of teledermatology and we will discuss system-level and practice-level barriers to successful teledermatology implementation as well as potential implications for dermatologists.

Kaposi Sarcoma Herpesvirus Inflammatory Cytokine Syndrome-like Clinical Presentation in Human Immunodeficiency Virus-infected Children in Malawi.

We describe 7 human immunodeficiency virus-infected Malawian children with Kaposi sarcoma who met criteria for Kaposi sarcoma herpesvirus (KSHV) inflammatory cytokine syndrome. Each presented with persistent fevers, bulky lymphadenopathy, massive hepatosplenomegaly, and severe cytopenias. Plasma analyses were performed in 2 patients, both demonstrating extreme elevations of KSHV viral load and interleukin 6.

Cost analysis of a store-and-forward teledermatology consult system in Philadelphia.

BACKGROUND: Store-and-forward (SAF) teledermatology (TD) has the potential to increase access to timely, high-quality care for underserved populations. However, the cost-effectiveness of TD for underserved populations is uncertain. OBJECTIVE: This study evaluates the potential cost savings associated with an SAF TD program implemented for an underserved population in the city health clinics of urban Philadelphia. METHODS: We performed a retrospective analysis of SAF TD consultations for 700 outpatients managed in 12 Philadelphia primary care clinics. Primary care providers were asked to specify a treatment plan, as well as the type of care for the patient, in the absence of the TD service. Analysis compared the cost of each patient case with use of the TD consult model versus with conventional care. RESULTS: In all, 27% of in-person dermatology clinic visits (189 of 700) and 3.29% of emergency room visits (23 of 700) were avoided by using TD. Compared with conventional care, TD had a mean expected cost savings of $10.00 to $52.65 per TD consult. In sensitivity analyses, these estimated savings remained positive across a range of parameters. LIMITATIONS: The cost analysis relies on several assumptions regarding the cost of care, and indirect costs were not included. CONCLUSION: TD can be a cost-saving model while increasing access to dermatologic care.

Teledermatology as a Means to Provide Multispecialty Care: A Case of Global Specialty Collaboration.

Telemedicine can serve as a platform for specialty collaboration and potentially address the lack of specialized and subspecialized care globally. In this article we present a case in which the use of teledermatology facilitated global collaboration between multiple specialists and subspecialists, resulting in high-quality care of a child from a remote area of Botswana. We present the lessons learned and factors that should be considered when engaging in global specialty collaboration, especially between developed and developing countries. We also discuss the potential limitations of telemedicine when used within a global context. With these considerations in mind, global specialty collaboration facilitated by telemedicine can provide a potential solution to the lack of access to specialized and subspecialized care.

Impact of store-and-forward (SAF) teledermatology on outpatient dermatologic care: A prospective study in an underserved urban primary care setting.

BACKGROUND: The clinical value of teledermatology in the primary care setting remains relatively unknown. OBJECTIVE: We sought to determine the impact of teledermatology on outpatient diagnosis, management, and access to dermatologic care in a resource-poor primary care setting. METHODS: We performed a prospective study of store-and-forward teledermatology consults submitted between January and November 2013 from 11 underserved clinics in Philadelphia to the University of Pennsylvania using mobile devices and the Internet. We assessed diagnostic and management concordance between primary care providers and dermatologists, time to consult completion, anticipated level of dermatology input in the absence of teledermatology, and number of consults managed with teledermatology alone. RESULTS: The study included 196 consults encompassing 206 dermatologic conditions. Diagnoses and management plans of primary care providers and dermatologists were fully concordant for 22% and 23% of conditions, respectively. The median time to consult completion was 14 (interquartile range 3-28) hours. At least 61% of consults would not otherwise have received dermatology input, and 77% of consults were managed with teledermatology alone. LIMITATIONS: Lack of a diagnostic gold standard, limited patient follow-up, and uncertain generalizability are limitations. CONCLUSION: Teledermatology is an innovative and impactful modality for delivering dermatologic care to outpatients in resource-poor primary care settings.

Tropical Skin Diseases in Children: A Review- Part I.

Because of travel and migration patterns, tropical skin diseases are now seen all around the world, not just in tropical or developing countries. Nutrition, housing, and environmental factors play an important role in these infectious diseases, so when they appear out of their normal environments, their classic presentation may vary. Tropical diseases can also present differently in childhood, making their recognition, diagnosis, and management a clinical challenge. Health care providers in developed countries need to be familiar with tropical skin diseases and be able to diagnose them in returning travelers or immigrants in order to optimize care. This article aims to review the epidemiologic, clinical, diagnostic, and therapeutic aspects of some of the most common tropical dermatologic conditions in children.

Tropical Skin Diseases in Children: A Review-Part II.

Tropical skin diseases are infectious conditions influenced by factors such as nutrition, housing, and the environment. Migration patterns have caused these conditions to be seen all around the world, not only in developing countries. Many of these diseases have a different presentation in childhood, which changes the diagnostic approach and management options. In this article, we review some of the most common tropical mycobacterial, protozoan, parasitic, and viral dermatologic conditions in children, including their epidemiologic, clinical, diagnostic, and therapeutic aspects.

Evaluation of a Mobile Health Approach to Tuberculosis Contact Tracing in Botswana.

Tuberculosis (TB) contact tracing is typically conducted in resource-limited settings with paper forms, but this approach may be limited by inefficiencies in data collection, storage, and retrieval and poor data quality. In Botswana, we developed, piloted, and evaluated a mobile health (mHealth) approach to TB contact tracing that replaced the paper form-based approach for a period of six months. For both approaches, we compared the time required to complete TB contact tracing and the quality of data collected. For the mHealth approach, we also administered the Computer System Usability Questionnaire to 2 health care workers who used the new approach, and we identified and addressed operational considerations for implementation. Compared to the paper form-based approach, the mHealth approach reduced the median time required to complete TB contact tracing and improved data quality. The mHealth approach also had favorable overall rating, system usefulness, information quality, and interface quality scores on the Computer System Usability Questionnaire. Overall, the mHealth approach to TB contact tracing improved on the paper form-based approach used in Botswana. This new approach may similarly benefit TB contact tracing efforts in other resource-limited settings.