Thomas M. Beachkofsky

Progressive Overgrowth of the Cerebriform Connective Tissue Nevus in Patients with Proteus Syndrome

BACKGROUND Proteus syndrome is a rare overgrowth disorder that almost always affects the skin. OBJECTIVE Our purpose was to evaluate progression of skin lesions in patients with Proteus syndrome. METHODS Skin findings were documented in 36 patients with Proteus syndrome. Progression of skin lesions in 16 of these patients was assessed by comparing photographs obtained on repeated visits for an average total duration of 53 months. RESULTS The skin lesion most characteristic of Proteus syndrome, the cerebriform connective tissue nevus, showed progression in 13 children but not in 3 adults. The cerebriform connective tissue nevus progressed by expansion into previously uninvolved skin, increased thickness, and development of new lesions. Lipomas increased in size, number, or both in 8 of 10 children with lipomas. In contrast, epidermal nevi and vascular malformations generally did not spread or increase in number. LIMITATIONS Only 3 adults with Proteus syndrome were evaluated longitudinally. CONCLUSION The cerebriform connective tissue nevus in Proteus syndrome grows throughout childhood but tends to remain stable in adulthood.

Adverse Events Following Smallpox Vaccination With ACAM2000 in a Military Population

BACKGROUND Generalized vaccinia and benign exanthems are 2 adverse events that have been associated with the smallpox vaccination. Accurate incidence and prevalence rates of each are not readily available, but these events are thought to be uncommon. To our knowledge, this is the first case series to provide clinical as well as pathologic descriptions of multiple papulovesicular eruptions occurring after receiving the second-generation smallpox vaccine, ACAM2000 (Acambis, Canton, Massachusetts), among a vaccinia-naïve military population. In addition, we report the first confirmed case, to our knowledge, of generalized vaccinia following administration of the ACAM2000 vaccine. OBSERVATIONS All patients received primary smallpox immunization as well as 1 to 3 concurrent or near-concurrent (within the preceding 21 days) immunizations for typhoid, anthrax, hepatitis B, and/or seasonal influenza. One patient presented with a flulike prodrome and diffuse vesiclopustules covering the face, neck, chest, back, and upper and lower extremities. Vaccinia polymerase chain reaction confirmed generalized vaccinia. The remaining 7 patients presented with unusual, painful, and pruritic papulovesicular eruptions occurring on the extensor surfaces of their upper and lower extremities without systemic symptoms. Histologic findings revealed 2 general patterns, including a dermal hypersensitivity reaction with lymphocytic vasculitis and a vesicular spongiotic dermatitis with eosinophils. CONCLUSIONS We present the first confirmed case of generalized vaccinia following immunization with the second-generation smallpox vaccine ACAM2000. In addition, we describe 7 cases of benign, acral, papulovesicular eruptions thought to be associated with ACAM2000 administration. Further research is needed to discern the pathogenesis of these benign eruptions as well as their incidence and prevalence and that of generalized vaccinia with ACAM2000.

Induction of De Novo Hair Regeneration in Scars After Fractionated Carbon Dioxide Laser Therapy in Three Patients

&NA; The authors have indicated no significant interest with commercial supporters.

SJS/TEN 2017: Building Multidisciplinary Networks to Drive Science and Translation

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) is a life-threatening, immunologically mediated, and usually drug-induced disease with a high burden to individuals, their families, and society with an annual incidence of 1 to 5 per 1,000,000. To effect significant reduction in short- and long-term morbidity and mortality, and advance clinical care and research, coordination of multiple medical, surgical, behavioral, and basic scientific disciplines is required. On March 2, 2017, an investigator-driven meeting was held immediately before the American Academy of Dermatology Annual meeting for the central purpose of assembling, for the first time in the United States, clinicians and scientists from multiple disciplines involved in SJS/TEN clinical care and basic science research. As a product of this meeting, this article summarizes the current state of knowledge and expert opinion related to SJS/TEN covering a broad spectrum of topics including epidemiology and pharmacogenomic networks; clinical management and complications; special populations such as pediatrics, the elderly, and pregnant women; regulatory issues and the electronic health record; new agents that cause SJS/TEN; pharmacogenomics and immunopathogenesis; and the patient perspective. Goals include the maintenance of a durable and productive multidisciplinary network that will significantly further scientific progress and translation into prevention, early diagnosis, and management of SJS/TEN.

Preliminary Efficacy Testing of the Disinfectant MicrobeCare XLP for Potential Use in Military Operational Environments

Introduction A safe, easy-to-use, permanently bonded antiseptic that does not require post-exposure bioload reduction but maintains effectiveness over time would have far-reaching implications across multiple industries. Health care is one such arena, particularly in austere military settings where resources are at a premium. MicrobeCare XLP (MicrobeCare, Buffalo Grove, IL, USA) is a commercially available spray-on agent that is advertised to covalently bond to surfaces and provide a long-lasting antimicrobial coating inhospitable to >99.99% of surface microorganisms. A pilot study was devised to gather baseline data regarding product efficacy and laboratory parameters before consideration of extended investigations and military utilization. The product manufacturer recommends bioload reductions before product application, following product application, and after each pathogenic exposure. To investigate the products efficacy in circumstances more closely simulating a military operational setting in which post-pathogenic exposure bioload reduction would not be possible, this step was deliberately excluded from the test sequences. Materials and Methods Using autoclaved surgical forceps, growth of Staphylococcus aureus and Acinetobacter baumannii was evaluated in a controlled manner under multiple conditions. Test variations included duration of submersion in the MicrobeCare XLP solution and air-drying and a second autoclave sterilization. Control and treated forceps were exposed to a bacterial suspension and air-dried before being submerged in sterile saline and vortex mixed. The saline solution was serially diluted and plated on tryptic soy agar (TSA) II plates. Plates were incubated for 24 h and bacterial colony-forming units (CFU)/mL were counted. Results Statistical significance was defined according to the American Society for Testing and Materials (ASTM) International passing criteria of 3 Log10 or 99.9% reduction of microorganisms. Additionally, p-values were calculated using two-tailed unpaired two-sample t-tests with unequal variance with a threshold of 0.05. In the S. aureus tests, none of the reduction calculations met the ASTM International passing criteria. In addition, the difference between the means of the colony counts in the MicrobeCare XLP-treated forceps and untreated control forceps was not statistically significant (p-value 0.109). Conversely, in the A. baumannii tests, each of the percent reduction calculations met the ASTM International passing criteria; the difference between the means of the colony counts in the treatment and control groups was statistically significant (p-value 0.008). Conclusion In these independent tests, MicrobeCare XLP effectively prevented growth of A. baumannii but had unpredictable results suppressing S. aureus. These results may relate to inherent properties of the bacteria or autoclave exposure, although the manufacturer asserts that the coating withstands such degradation. Additional testing could be performed using a broader range of microorganisms and exposure to varying conditions including other sterilization methods.

CYP2C19*2 status in patients with Stevens-Johnson syndrome and toxic epidermal necrolysis

Purpose Genetic polymorphisms have been linked to an increased predisposition to developing certain diseases. For example, patients of Han-Chinese descent carrying the HLA-B*1502 allele are at an increased risk of developing Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) if given carbamazepine. Given the complexity of in vivo drug metabolism, it is plausible that the activity of enzyme systems unrelated to specific drug metabolism may be important. Although multiple biomarkers have been identified in unique ethnic groups, there has yet to be a study investigating the presence of the slow metabolizing allele of CYP2C19, denoted CYP2C19*2, in diverse groups and the risk of developing SJS/TEN. Patients and methods This study looked into the carrier status of CYP2C19*2, a poor metabolizing variant of CYP2C19, in patients diagnosed with SJS/TEN. We looked at its status in our series as a whole and when patients were divided by ethnicity. Genomic DNA was extracted from formalin-fixed paraffin-embedded tissue of patients with biopsy-proven SJS/TEN and real-time polymerase chain reaction was used to assess for the presence of CYP2C19*2. Results CYP2C19*2 status was determined in 47 patients. Twenty-nine of these 47 patients had a single medication implicated as causing their disease, and eight of these patients were heterozygous or homozygous for CYP2C19*2. There was insufficient evidence to conclude that the presence of CYP2C19*2 is an independent predictor of risk for developing SJS/TEN in our series as a whole. This analysis also confirmed that the frequency of the CYP2C19*2 polymorphism within the different ethnicities in our series did not vary statistically from reported ethnic rates. Conclusion Our study was unable to show a relationship between CYP2C19*2 status and predisposition toward SJS/TEN. We had a heterogeneous population, making it difficult to control for possible confounding factors.