Posttraumatic onychoheterotopia: A case and review of the pediatric literature.

BACKGROUND: Onychoheterotopia, or ectopic nail, is a rare nail condition in which nail growth occurs in areas other than the distal phalanges. The majority of cases in the literature are noted to be congenital; however, acquired cases have less commonly been described following either acute or chronic trauma. METHODS: We present a case of acquired onychoheterotopia in a child, with a corresponding literature review of acquired pediatric onychoheterotopia cases for comparison. RESULTS: There were a total of 10 cases of acquired onychoheterotopia in pediatric patients in the literature. Including our patient, 81.8% (9/11) developed an ectopic nail on the fingers. Crush injury preceded the ectopic nails in 45.5 % (5/11) of cases. Infection occurred in 27.3% (3/11). CONCLUSIONS: Although the incidence and prevalence of acquired onychoheterotopia are unknown, it seems to be an uncommon phenomenon. From our case and others from the literature, there may be associations with crush injury specifically. Further research will be needed to fully elucidate the pathophysiology and risk factors for ectopic nail growth.

Acral Changes in pediatric patients during COVID 19 pandemic: Registry report from the COVID 19 response task force of the society of pediatric dermatology (SPD) and pediatric dermatology research alliance (PeDRA).

BACKGROUND/OBJECTIVE: In spring 2020, high numbers of children presented with acral pernio-like skin rashes, concurrent with the coronavirus disease 2019 (COVID-19) pandemic. Understanding their clinical characteristics/ infection status may provide prognostic information and facilitate decisions about management. METHODS: A pediatric-specific dermatology registry was created by the Pediatric Dermatology COVID-19 Response Task Force of the Society for Pediatric Dermatology (SPD) and Pediatric Dermatology Research Alliance (PeDRA) and was managed by Children's Hospital of Philadelphia using REDCap. RESULTS: Data from 378 children 0-18 years entered into the registry between April 13 and July 17, 2020 were analyzed. Data were drawn from a standardized questionnaire completed by clinicians which asked for demographics, description of acral lesions, symptoms before and after acral changes, COVID-19 positive contacts, treatment, duration of skin changes, laboratory testing including SARS-CoV-2 PCR and antibody testing, as well as histopathology. 229 (60.6%) were male with mean age of 13.0 years (± 3.6 years). Six (1.6%) tested positive for SARS-CoV-2. Pedal lesions (often with pruritus and/or pain) were present in 96%. 30% (114/378) had COVID-19 symptoms during the 30 days prior to presentation. Most (69%) had no other symptoms and an uneventful course with complete recovery. CONCLUSIONS AND RELEVANCE: Children with acral pernio-like changes were healthy and all recovered with no short-term sequelae. We believe these acral changes are not just a temporal epiphenomenon of shelter in place during the spring months of the first wave of the COVID-19 pandemic and may be a late phase reaction that needs further study.

Clustered cases of acral perniosis: Clinical features, histopathology, and relationship to COVID-19.

BACKGROUND/OBJECTIVES: A recent marked increase in pediatric and adult patients presenting with purpuric acral lesions concerning for ischemia, thrombosis and necrosis has been observed in COVID-19 prevalent regions worldwide. The clinical and histopathological features and relationship to COVID-19 have not been well described. The objective of this case series is to describe the clinical features and determine the histopathologic findings and clinical implications of the clusters of acral perniosis cases identified in pediatric patients. METHODS: We describe six otherwise healthy adolescents-three siblings per family from two unrelated families-presented within a 48-hour period in April, 2020, with acral perniosis-like lesions in the context of over 30 similar patients who were evaluated within the same week. RESULTS: Affected patients had mild symptoms of viral upper respiratory infection (URI) or contact with symptomatic persons 1-2 weeks preceding the rash. They all presented with red to violaceous macules and dusky, purpuric plaques scattered on the mid and distal aspects of the toes. Skin biopsies performed on each of the six patients demonstrated near identical histopathologic findings to those of idiopathic perniosis, with a lymphocytic inflammatory infiltrate without evidence of thromboembolism or immune complex vasculitis. While SARS-CoV-2 polymerase chain reaction was negative, testing was performed 1-2 weeks after URI symptoms or sick contact exposure. CONCLUSION: We offer a clinical approach to evaluation of patients with this presentation and discuss the possibility that these skin findings represent a convalescent-phase cutaneous reaction to SARS-CoV-2 infection.

Systemic immunosuppressive therapy for inflammatory skin diseases in children: Expert consensus-based guidance for clinical decision-making during the COVID-19 pandemic.

BACKGROUND/OBJECTIVES: The COVID-19 pandemic has raised questions about the approach to management of systemic immunosuppressive therapies for dermatologic indications in children. Change to: Given the absence of data to address concerns related to SARS-CoV-2 infection and systemic immunosuppressive therapies in an evidence-based manner, a Pediatric Dermatology COVID-19 Response Task Force (PDCRTF) was assembled to offer time-sensitive guidance for clinicians. METHODS: A survey was distributed to an expert panel of 37 pediatric dermatologists on the PDCRTF to assess expert opinion and current practice related to three primary domains of systemic therapy: initiation, continuation, and laboratory monitoring. RESULTS: Nearly all respondents (97%) reported that the COVID-19 pandemic had impacted their decision to initiate immunosuppressive medications. The majority of pediatric dermatologists (87%) reported that they were pausing or reducing the frequency of laboratory monitoring for certain immunosuppressive medications. In asymptomatic patients, continuing therapy was the most popular choice across all medications queried. The majority agreed that patients on immunosuppressive medications who have a household exposure to COVID-19 or test positive for new infection should temporarily discontinue systemic and biologic medications, with the exception of systemic steroids, which may require tapering. CONCLUSIONS: The ultimate decision regarding initiation, continuation, and laboratory monitoring of immunosuppressive therapy during the pandemic requires careful deliberation, consideration of the little evidence available, and discussion with families. Consideration of an individual's adherence to COVID-19 preventive measures, risk of exposure, and the potential severity if infected must be weighed against the dermatological disease, medication, and risks to the patient of tapering or discontinuing therapies.

How issues of autonomy and consent differ between children and adults: Kids are not just little people.

Decision making in dermatology practice with adult patients is in most cases a relatively straightforward process, involving a back-and-forth exchange of information, ideas and opinions between the physician and patient. This process is more complex in the field of pediatric dermatology. It involves the triad of the patient, parent or guardian, and physician. It utilizes a model, common to many fields in pediatrics, whereby the physician seeks to obtain informed permission from the parent with the assent of the child, all the while endeavoring to act in the best interests of the child. This model can be challenged in many ways; from parents seeking interventions, which are not in the best interests of the child, to a child refusing to assent to a procedure that is deemed necessary by the parent and physician. We review the theory, terminology, and guidelines relevant to the ethics of decision making when working with children compared with adults. We explore approaches to a variety of ethically challenging situations that may be encountered by the dermatologist, through their work with pediatric patients.

Adherence of Tc-99 Sestamibi to Plastic Syringes Could Complicate Efforts in Dose Reduction in MPI SPECT.

BACKGROUND: Multiple disposable syringe designs are available both with and without silicone lubricant. Tc99m sestamibi variably adheres to certain syringe designs. In practice, this increases pre-injection activity to ensure constant acceptable image quality in the setting of high adherence. METHODS: The residual activity following 35 Tc99m MDP injections was used as a proxy for the dead-space residual activity. This was compared to Tc99m sestamibi injections in the same syringe in 107 injections, a syringe without lubricant in 35 injections, and a syringe without lubricant or rubber gasket in 16 injections. The syringe with lubricated barrel was disassembled and each part separately assayed to see which part demonstrated the most adherence. RESULTS: The standard design demonstrated a mean adherence of 26% (SD 7.0%). This was significantly more than the 6% (SD 3.7%) demonstrated with MDP. The two designs without silicone lubricant demonstrated mean adherence of 7.2% (SD 2.3%) and 6.4% (SD 2.3%). Disassembly of the three part syringe showed the greatest adherence to the rubber gasket. CONCLUSIONS: QC procedures determining adherence in the administration syringe are warranted for any change in syringe design or manufacturer. If adherence is present, conversion to non-lubricated plastic syringes with lower and more predictable retention of Tc99m sestamibi enables more precise titration of prescribed activities and reduction of overall patient absorbed doses.