Background/ObjectivesThe 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.MethodsA 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.ResultsNearly 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.ConclusionsThe 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.
We describe a case of linear porokeratosis with associated bone resorption in a 17-year-old female with marked improvement after 2% cholesterol/2% lovastatin ointment application. Porokeratosis is a heterogenous group of keratinization disorders characterized by a cornoid lamella, consisting of focal dyskeratotic cells in the granular layer and columns of parakeratosis. The pathogenesis of porokeratosis is not fully elucidated; however, germline mutations have recently been identified in the mevalonate pathway which can lead to a buildup of metabolites that could play a role in dysmaturation. There has only been one prior report of an affected distal digit with underlying bone resorption in association with linear porokeratosis.
Background: The location of telangiectases in hereditary hemorrhagic telangiectasia (HHT), as set forth in the consensus diagnostic (Curaçao) criteria, is based primarily on adults.Objective: Document the locations and numbers of telangiectases in a cohort of pediatric patients with HHT.Methods: A retrospective chart review using a standardized data collection form for site and number of telangiectases was performed for pediatric patients with HHT (age, 0-18 years) from 2005 to 2016.Results: Of 90 pediatric patients with HHT, 71% had one or more telangiectases. Of all the telangiectases counted (N = 319), cutaneous telangiectases were more common (73%) than oral telangiectases (27%). The hands were the most frequent site, accounting for 33% of all telangiectases. Adolescents were more likely than children to have cutaneous telangiectases (85% vs 50% [Q = 0.005]). The most frequent sites in children younger than 10 years were the hands excluding the fingers (27%), fingers (25%), and face (23%). Only 23% of subjects (21 of 90) presented with multiple ($3) telangiectases at locations considered characteristic for the current consensus diagnosis guidelines (lips, oral cavity, and fingers).Limitations: Ascertainment bias based on recruitment.
Conclusions:In this pediatric population, telangiectases at sites not included as ''characteristic'' by the Curaçao diagnostic criteria were common. The Curaçao criteria in regard to both number and location of telangiectases may be inadequate in the pediatric HHT population.
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