Classification of lower extremity venous malformations and risk of knee involvement: A retrospective cohort study.

BACKGROUND: Venous malformation (VM) is the most common vascular anomaly in the lower extremity. VMs can be classified as focal, multifocal, or diffuse types. Intraarticular VM (IA-VM) of the knee portends morbidity. Association of the lower extremity VM type with IA-VM is not well defined. OBJECTIVE: To classify a large cohort of lower extremity, nonsyndromic VMs by type and determine associations with IA-VM. METHODS: Retrospective cohort study. RESULTS: We assessed 156 patients with nonsyndromic, lower extremity VM; 71 (46%) were focal and 85 (54%) were diffuse type VM, and 97 (62%) were IA-VM. Of diffuse VMs, 26 (31%) were Bockenheimer and 59 (69%) were localized subtypes. Pure VM had a significantly elevated risk of IA-VM (relative risk [RR], 2.34; 95% confidence interval [CI], 1.42-3.89). IA-VM was more common in diffuse (73%) versus focal (49%) types. Risk of IA-VM in diffuse type VM was significantly elevated (RR, 1.48; 95% CI, 1.13-1.94). One hundred percent of diffuse Bockenheimer type VM had IA-VM, and this subtype had the highest risk (RR, 1.83; 95% CI, 1.56-2.14) of IA-VM. LIMITATIONS: Retrospective, single-institution study. CONCLUSIONS: Intraarticular involvement of the knee should be considered in all lower extremity VMs. Pure VM and the Bockenheimer diffuse VM subtype had the highest risk of IA-VM.

Infantile toxic epidermal necrolysis: Successful treatment of an 8-week-old with intravenous immunoglobulin and amniotic membrane transplant.

Stevens-Johnson syndrome and toxic epidermal necrolysis comprise a spectrum of severe mucocutaneous hypersensitivity reactions. A paucity of data limits current understanding of the etiology, treatment options, and prognosis of this entity in the infantile population compared to that in the adult and pediatric literature. We describe the case of an 8-week-old male with toxic epidermal necrolysis treated successfully with intravenous immunoglobulin and amniotic membrane transplant. This patient is the youngest surviving infant with toxic epidermal necrolysis to be reported.

Pemphigus-like eruption as a complication of molluscum contagiosum treatment with imiquimod in a 5-year-old girl.

Pemphigus and pemphigus-like reactions can be triggered by a variety of medications including topical therapies, such as imiquimod. While the association between imiquimod and pemphigus-like reactions has been reported in adults, this is the first report of a generalized reaction beyond the site of imiquimod application in a child. The mechanism by which this occurs may be through a unique pathway, separate from the classic antibody-mediated pathway. Our patient had a full recovery without recurrence after cessation of the inciting drug.

Adams-Oliver Syndrome Type 2 in Association with Compound Heterozygous DOCK6 Mutations.

Adams-Oliver syndrome (AOS) is a multiple congenital anomaly syndrome characterized by aplasia cutis congenita (ACC) and transverse terminal limb defects (TTLDs). We present a case of type 2 autosomal recessive AOS associated with heterozygous mutations in the dedicator of cytokinesis 6 (DOCK6) gene, with characteristic findings of ACC, TTLD, intracerebral periventricular calcifications, and polymicrogyria.

Role of cytokine hemoadsorption in cardiopulmonary bypass-induced ventricular dysfunction in a porcine model.

Little is known about the effect of cardiopulmonary bypass alone on cardiac function; in an attempt to illuminate this relationship and test a possible mechanism, we used Cytosorb, a device capable of removing virtually all types of circulating cytokines to test the hypothesis that hemoadsorption of cytokines during bypass attenuates bypass-induced acute organ dysfunction. Twelve Yorkshire pigs (50-65 kg) were instrumented with a left ventricular conductance catheter. Baseline mechanics and cytokine expression (tumor necrosis factor [TNF], interleukin-6 [IL-6], and interleukin-10) were measured before and hourly after 1 hour of normothermic cardiopulmonary bypass. Animals underwent bypass without (cardiopulmonary bypass [CPB], n = 6) or with (CPB+HA, n = 6) the CytosorbTM device. Data were compared with "historical" controls (n = 6) that were similarly instrumented but underwent observation instead of bypass. Five hours after separation from bypass (or observation), animals were euthanized. Myocardial water content was determined postmortem. Neither TNF nor IL-6 was significantly elevated in either experimental group versus controls at any time point. Preload recruitable stroke work and dP/dtmax were significantly depressed immediately after separation from bypass in both CPB+HA and CPB and remained depressed for the duration of the experiment. Although Tau remained unchanged, dP/dTmin was significantly diminished in both bypass groups at all time points after separation from bypass. Cytokine hemoadsorption had no effect on any measurable index of function. Differences in postmortem data were not evident between groups. One hour of normothermic CPB results in a significant and sustained decline in left ventricular function that appears unrelated to changes in cytokine expression. Because we did not appreciate a significant change in cytokine concentrations postbypass, the capacity of cytokine hemoadsorption to attenuate CPB-induced ventricular dysfunction could not be assessed.

Going viral: A brief history of Chilblain-like skin lesions ("COVID toes") amidst the COVID-19 pandemic.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the cause of the COVID-19 global pandemic, is notable for an expanding list of atypical manifestations including but not limited to coagulopathies, renal dysfunction, cardiac injury and a multisystem inflammatory syndrome in children. In addition, SARS-CoV-2 has been purportedly linked to multiple cutaneous manifestations, among them chilblain-like skin lesions, also known as "COVID toes." Driven in large part by social media, dermatologists around the world reported a dramatic increase in the frequency of chilblain-like diagnoses early in the COVID-19 pandemic, often in members of the same family. This phenomenon has been captured in a rapidly expanding medical literature. As of this writing, the chilblain-like presentation has been reported to occur predominantly in younger, minimally symptomatic patients and to emerge late in the COVID-19 disease course. Evidence of SARS-CoV-2 infection is not consistently found when these patients are evaluated by polymerase chain reaction. A robust antiviral immune response in young patients that induces microangiopathic changes has been posited as a mechanism. Herein we review the rapid evolution of the literature regarding chilblain-like skin lesions early in the COVID-19 global pandemic.

Successful Use of the 308-nm Excimer Laser in Early Patch Stage Mycosis Fungoides.

Introduction: Mycosis fungoides (MF), a type of cutaneous T-cell lymphoma, is a rare condition with a variety of treatment options. A frequently utilized method in the treatment of early-stage MF is phototherapy. Full body phototherapy can be associated with photoaging of the skin and an increased risk of developing skin cancer. Targeted phototherapy, with a 308-nm excimer laser, may be just as effective and of a lower risk. This makes it especially useful in the treatment of patients with dysplastic nevi (DN) or other conditions which can put them at a higher risk of developing skin cancer. There are currently limited data on the treatment of early-stage MF with an excimer laser. Case Report: This study reports on a 43-year-old female patient presented to the clinic with early-stage (Ia) MF. Given her history of DN, she wished to pursue targeted phototherapy with an excimer laser. Localized light treatment was performed via a 308-nm excimer laser, 3 times weekly, for a total of 17 treatments to affected lesions. Following excimer laser treatment, she had a clinical resolution of her patches. On the follow-up clinic visits, she maintained her excellent response 12 months out. Conclusion: Targeted phototherapy with a 308-nm excimer laser may be a safer and equally effective alternative to generalized phototherapy in the treatment of early-stage MF. This case report demonstrates its efficacy and advantages over traditional generalized phototherapy.