Acute rheumatic fever and rheumatic heart disease among children--American Samoa, 2011-2012.

Acute rheumatic fever is a nonsuppurative, immune-mediated consequence of group A streptococcal pharyngitis (strep throat). Recurrent or severe acute rheumatic fever can cause permanent cardiac valve damage and rheumatic heart disease, which increases the risk for cardiac conditions (e.g., infective endocarditis, stroke, and congestive heart failure). Antibiotics can prevent acute rheumatic fever if administered no more than 9 days after symptom onset. Long-term benzathine penicillin G (BPG) injections are effective in preventing recurrent acute rheumatic fever attacks and are recommended to be administered every 3-4 weeks for 10 years or until age 21 years to children who receive a diagnosis of acute rheumatic fever. During August 2013, in response to anecdotal reports of increasing rates of acute rheumatic fever and rheumatic heart disease, CDC collaborated with the American Samoa Department of Health and the Lyndon B. Johnson Tropical Medical Center (the only hospital in American Samoa) to quantify the number of cases of pediatric acute rheumatic fever and rheumatic heart disease in American Samoa and to assess the potential roles of missed pharyngitis diagnosis, lack of timely prophylaxis prescription, and compliance with prescribed BPG prophylaxis. Using data from medical records, acute rheumatic fever incidence was calculated as 1.1 and 1.5 cases per 1,000 children aged ≤18 years in 2011 and 2012, respectively; 49% of those with acute rheumatic fever subsequently received a diagnosis of rheumatic heart disease. Noncompliance with recommended prophylaxis with BPG after physician-diagnosed acute rheumatic fever was noted for 22 (34%) of 65 patients. Rheumatic heart disease point prevalence was 3.2 cases per 1,000 children in August 2013. Establishment of a coordinated acute rheumatic fever and rheumatic heart disease control program in American Samoa, likely would improve diagnosis, treatment, and patient compliance with BPG prophylaxis.

Prevaccine era human papillomavirus types 6, 11, 16 and 18 seropositivity in the U.S.A., National Health and Nutrition Examination Surveys, 2003-2006.

BACKGROUND: A vaccine is available to prevent human papillomavirus (HPV) 6, 11, 16 and 18; in the prevaccine era, seropositivity to vaccine types is a measure of natural exposure. METHODS: We describe HPV seropositivity in the USA among 14-59-year-olds using the 2003-2006 National Health and Nutrition Examination Surveys. RESULTS: Seropositivity to HPV 6, 11, 16 and 18 was 17.5%, 6.8%, 15.1% and 5.9%, respectively, among women, and 7.0%, 2.4%, 5.2% and 1.5%, respectively, among men. Overall in both sexes, seropositivity was 22.5% for any vaccine type (31.8% in women and 12.9% in men), but substantially lower for three or more types (1.7% overall, 2.8% in women and 0.6% in men). CONCLUSIONS: Almost a quarter of the participants were seropositive to any HPV vaccine type but few were seropositive to at least three vaccine HPV types in the prevaccine era. Further study is needed to assess if seropositivity would be useful as a biological marker of vaccination.

Congenital syphilis investigation processes and timing in Louisiana.

BACKGROUND: Congenital syphilis (CS) is a potentially life-threatening yet preventable infection. State and local public health jurisdictions conduct investigations of possible CS cases to determine case status and to inform public health prevention efforts. These investigations occur when jurisdictions receive positive syphilis test results from pregnant women or from infants. METHODS: We extracted data from Louisiana's electronic case management system for 328 infants investigated as possible CS cases in 2010 to 2011. Using date stamps from the case management system, we described CS investigations in terms of processes and timing. RESULTS: Eighty-seven investigations were prompted by positive test results from women who were known to be pregnant by the health jurisdiction, and 241 investigations were prompted by positive syphilis test results from infants. Overall, investigations required a median of 101 days to complete, although 25% were complete within 36 days. Investigations prompted by positive test results from infants required a median of 135 days to complete, and those prompted by positive test results from pregnant women required a median of 41 days. CONCLUSIONS: Three times as many CS investigations began with reported positive syphilis test results from infants as from pregnant women, and these investigations required more time to complete. When CS investigations begin after an infant's birth, the opportunity to ensure that women are treated during pregnancy is missed, and surveillance data cannot inform prevention efforts on a timely basis. Consistently ascertaining pregnancy status among women whose positive syphilis test results are reported to public health jurisdictions could help to assure timely CS prevention efforts.

Zoonotic Majocchi's Granuloma of the Vulva.

A healthy 32-year-old woman presented to clinic with tender pruritic lesions of 2-month duration at the vulva and lesions for weeks on the shins. She was treated with topical corticosteroids and intravenous vancomycin without significant improvement. On examination, dozens of follicular hemorrhagic papulopustules were detected at the suprapubic area and vulva (Figure 1). Similar but less prominent lesions were observed on the shins as well. Biopsies of the vulva and shin revealed a follicular inflammatory infiltrate of neutrophils, histiocytes, and lymphocytes as well as fungal hyphae within the follicular infundibulum and hair shafts, consistent with Majocchi's granuloma (MG). Gram and Fite-Faraco staining, direct immunofluorescence, and bacterial culture were negative. Tissue culture grew Trichophyton mentagrophytes, which was identified using sequence analysis of the D1/D2 region of the 28s rDNA. Minimum inhibitory concentrations for terbinafine, ketoconazole, and itraconazole were determined, with terbinafine having the lowest concentration. Additional history revealed that shortly prior to commencement of her clinical manifestations, the patient had acquired a pet guinea pig with eruptions and hair loss (Figure 2). The patient was prescribed ketoconazole cream and terbinafine, 250 mg daily, with almost immediate improvement. Based on clinical response, the patient remained on terbinafine and ketoconazole cream for 6 months. Her skin remained clear 4 months after discontinuing all antifungals. Based on the results of patient's culture, a veterinarian treated her guinea pig successfully with systemic terbinafine and miconazole lotion.

Hospitalization cost of congenital syphilis diagnosis from insurance claims data in the United States.

We analyzed medical insurance claims data (2005-2009) to determine the hospitalization costs of newborns with congenital syphilis (CS) diagnoses. We found 44 admission claims with CS diagnosis. The excess cost per case of CS (in 2009 US dollars) was estimated at $9969 (95% confidence interval, $5702-$16,769).

Prevalence of circumcision among men and boys aged 14 to 59 years in the United States, National Health and Nutrition Examination Surveys 2005-2010.

BACKGROUND: In 2009, an estimated 3590 new heterosexually acquired HIV infections occurred in males in the United States. Three randomized controlled trials demonstrated that male circumcision decreased a man's risk for HIV acquisition through heterosexual sex. We describe circumcision prevalence in US males and determine circumcision prevalence among males potentially at increased risk for heterosexually acquired HIV infection. METHODS: We estimated circumcision prevalence among men and boys aged 14 to 59 years using data from the National Health and Nutrition Examination Surveys 2005-2010. We defined men and boys with 2 or more female partners in the last year as potentially at increased risk for heterosexually acquired HIV infection. RESULTS: Estimated circumcision prevalence was 80.5%. Prevalence varied significantly by year of birth, race/ethnicity, health insurance type, and family income. Circumcision prevalence among men and boys reporting 2 or more female partners in the last year was 80.4%, which corresponded to an estimated 3.5 million uncircumcised men and boys potentially at increased risk for heterosexually acquired HIV infection. Of these men and boys, 48.3% lacked health insurance. CONCLUSIONS: Circumcision prevalence in the United States differs by demographic group, and half of uncircumcised men and boys potentially at increased risk for heterosexually acquired HIV are uninsured. These data could inform recommendations and cost analyses concerning circumcision in the United States.

Expedited partner therapy in federally qualified health centers--New York City, 2012.

BACKGROUND: Management of patients' sex partners is a critical element of sexually transmitted disease (STD) control. Expedited partner therapy (EPT), a practice in which patients deliver medication or a prescription directly to their partners, is one option for partner management. As of 2009, New York State law specifically allows EPT for chlamydial infection. Federally qualified health centers (FQHCs) in New York City (NYC) care for patients at risk for STDs. We describe the policies and practices surrounding EPT and other STD management in NYC FQHCs. METHODS: In 2012, we surveyed medical directors at all NYC FQHC parent entities and clinicians at a sample of their corresponding clinical sites about written policies and actual practices regarding EPT for chlamydial infection and other STD management. RESULTS: Twenty-two entities (22/29; 76%) and 51 sites (51/72; 70%) responded to the survey. More than half of entities have a written policy permitting EPT, and 80% of sites provide EPT. Most entity policies allow EPT for, and most sites provide EPT to, adolescents and adults with both opposite-sex and/or same-sex partners. Most sites use electronic health records and provide EPT by prescriptions, and one third of sites do not provide educational materials with EPT. CONCLUSIONS: Our results indicate widespread EPT provision by NYC FQHCs; however, areas for improvement exist, specifically in following guidelines that recommend providing educational materials with EPT and do not recommend EPT for men with male partners. The use of prescriptions for EPT and electronic health records were identified as potential barriers to EPT provision.

Challenges in congenital syphilis surveillance: how are congenital syphilis investigations classified?

BACKGROUND: Congenital syphilis is a serious, preventable, and nationally notifiable disease. Despite the existence of a surveillance case definition, congenital syphilis is sometimes classified differently using an algorithm on the Centers for Disease Control and Prevention's case reporting form. METHODS: We reviewed Louisiana's congenital syphilis electronic reporting system for investigations of infants born from January 2010 to October 2011, abstracted data required for classification, and applied the surveillance definition and the algorithm. We calculated the sensitivities and specificities of the algorithm and Louisiana's classification using the surveillance definition as the surveillance gold standard. RESULTS: Among 349 congenital syphilis investigations, the surveillance definition identified 62 cases. The algorithm had a sensitivity of 91.9% and a specificity of 64.1%. Louisiana's classification had a sensitivity of 50% and a specificity of 91.3% compared with the surveillance definition. CONCLUSIONS: The differences between the algorithm and the surveillance definition led to misclassification of congenital syphilis cases. The algorithm should match the surveillance definition. Other state and local health departments should assure that their reported cases meet the surveillance definition.

Improvement in peripheral blood disease burden in patients with Sézary syndrome and leukemic mycosis fungoides after total skin electron beam therapy.

BACKGROUND: There is a paucity of effective therapies for patients with Sézary syndrome and advanced mycosis fungoides with peripheral blood involvement. Total skin electron beam (TSEB) radiation therapy is an extremely effective skin-directed therapy for these patients, but, until recently, it was thought not to signifcantly affect the peripheral blood malignant T-cell population. OBJECTIVE: We conducted this study to determine if TSEB has therapeutic effect on the peripheral blood in patients with advanced mycosis fungoides and Sézary syndrome. METHODS: All patients on stable medication regimens seen in our photopheresis facility who received TSEB therapy between January 2008 and October 2011 at Temple University Hospital, Philadelphia, PA, were analyzed retrospectively for improvement in the peripheral blood, as documented by flow cytometry. RESULTS: Six of 11 patients achieved 50% or greater decrease in their peripheral blood malignant T-cell population after TSEB therapy, for an overall response rate of 55%. Within the group of patients who had a response in the skin, 67% also had a response in the peripheral blood. LIMITATIONS: This analysis is limited in 3 ways. First, the sample described is small. Second, the results may be confounded by the fact that each patient was on other systemic therapies in addition to TSEB, albeit stable pre-existing regimens. The time interval between completion of TSEB therapy and repetition of flow cytometry was not standardized among patients, which may result in an underestimation of the overall response to TSEB therapy. CONCLUSION: In patients with advanced mycosis fungoides and Sézary syndrome, the peripheral blood tumor burden may improve after treatment with TSEB.

Blue Papules in a Child: Family History Might Prevent Diagnostic Tears.

A healthy 14-month-old boy presented with firm and grouped blue papules in the right mid area of the back and the right peri-anal area (Figure 1). The lesions first appeared when the boy was a 6- or 9-month old, gradually increasing in number to a total of six lesions. The child was delivered full-term with no complications, had no medical issues, and no personal or family history of gastrointestinal bleeding or anemia.

Acquired Lymphangiectasia in the Setting of a Gastrointestinal Stromal Tumor.

A 53-year-old woman presented for a skin cancer screening and was found to have scattered violaceous papulovesicles on the right buttock and right lower aspect of the abdomen. These lesions had been increasing in number for 3 years. The patient denied pain or pruritis at the site of lesions and denied any systemic fi ndings.

Validity assessment of the cutaneous T-cell lymphoma severity index to predict prognosis in advanced mycosis fungoides/Sézary syndrome.

BACKGROUND: There is a need for standardized quantitative disease assessment measures in mycosis fungoides/Sézary syndrome. In 2005, a cutaneous T-cell lymphoma (CTCL)-severity index (SI) that not only measures disease extent (on a scale of 0-75) independent of the classic TNM(B) staging system but can also be used to estimate individual 5-year survival (SR5) was reported. OBJECTIVE: We sought to assess the generalizability of the CTCL-SI/SR5 equation (SR5 equation) to predict prognosis in our cohort of patients with advanced mycosis fungoides/Sézary syndrome (n = 50, photopheresis service, 1984-2001). METHODS: TNM(B) staging, CTCL-SI score (based on skin involvement, presence of tumors, lymph node/visceral/blood involvement), and SR5 (SR5 equation = 124 - 2 × [CTCL-SI]%) at initial diagnosis were calculated retrospectively and compared with overall survival by the Kaplan-Meier method. The prognostic significance of TNM(B) staging versus the CTCL-SI was determined by Cox proportional hazards models and Brier scores. RESULTS: Patients had stage IIA to IVA disease with a median actuarial overall survival of 58 months. By disease stage, the overall 5-year survival was 70% (stage IIA), 48% (stage IIB-IIIB), and 36% (stage IVA). In our cohort, the CTCL-SI itself was predictive of overall survival (P = .028) but the SR5 equation was not predictive of survival (Brier score of 0.29). LIMITATIONS: Small sample size, single academic center population, and retrospective design are limitations. CONCLUSIONS: The CTCL-SI is a relatively simple-to-use quantitative tool that measures disease activity in all compartments (skin, nodes, blood, viscera) and has prognostic significance in multivariate analysis. The CTCL-SI may be a useful adjunct to the TNM(B) staging for tracking disease activity quantitatively in all disease compartments (skin, nodes, blood, viscera) in clinical practice and trials, but the predictive ability of the SR5 equation needs further validation at other centers in larger groups of patients.

Cutaneous toxicities of antiretroviral therapy for HIV: part I. Lipodystrophy syndrome, nucleoside reverse transcriptase inhibitors, and protease inhibitors.

Antiretroviral medications for the treatment of HIV are common drugs with diverse and frequent skin manifestations. Multiple new cutaneous effects have been recognized in the past decade. Dermatologists play an important role in accurately diagnosing and managing the cutaneous toxicities of these medications, thereby ensuring that a patient has as many therapeutic options as possible for life-long viral suppression. Part I of this two-part series on the cutaneous adverse effects of antiretroviral medications will discuss HIV-associated lipodystrophy syndrome, which can be seen as a result of many antiretroviral medications for HIV, and the specific cutaneous effects of the nucleoside reverse transcriptase inhibitors and protease inhibitors.

Cutaneous toxicities of antiretroviral therapy for HIV: part II. Nonnucleoside reverse transcriptase inhibitors, entry and fusion inhibitors, integrase inhibitors, and immune reconstitution syndrome.

Cutaneous manifestations of antiretroviral medications for HIV are common and potentially dangerous conditions encountered by dermatologists. Part II of this two-part series on the cutaneous effects of antiretroviral medications for HIV will discuss the four most recent classes of medications that have been developed and immune reconstitution syndrome--an important diagnostic consideration when evaluating a dermatologic patient who is taking antiretroviral medications.

A novel regimen of vorinostat with interferon gamma for refractory Sézary syndrome.

Long-term prognosis for advanced stages of cutaneous T-cell lymphoma may be beneficially altered with the use of multimodality therapy. However, refractory disease exists in which current therapeutic options fail to halt the progression of disease. We present 3 cases of refractory Sézary syndrome in which the combination of vorinostat and interferon gamma was well tolerated and produced significant clinical improvement. The potential immunologic basis for this is discussed.

Cutaneous Hodgkin's disease.

Cutaneous Hodgkin's disease is a rare condition that usually occurs late in the course of Hodgkin's lymphoma. This rare condition is thought to have decreased in incidence in recent decades, likely owing to improved treatment of patients with Hodgkin's disease, who are receiving improved chemotherapy and radiation therapy, and the advent of peripheral blood stem cell transplantation. We present the case of a man who developed specific cutaneous Hodgkin's lymphoma 6 months after nonmyeloablative allogenic stem cell transplantation for his recurrent systemic disease. The patient's manifestation of relapse was cutaneous dissemination of the tumor, manifested by erythematous papules and ulcerated nodules. At the time of the cutaneous relapse he had minimal systemic disease. This case illustrates an example of this complication of Hodgkin's disease and stresses the importance of a timely diagnosis to direct appropriate therapy. A review of the literature demonstrates that the patient's lesion morphology and distribution are typical of specific manifestations of cutaneous Hodgkin's disease.

Stem cell transplantation in advanced cutaneous T-cell lymphoma.

Cutaneous T-cell lymphomas are a type of indolent non-Hodgkin's lymphoma where patients with limited skin disease can be successfully treated with a variety of skin-directed, systemic, and immunomodulating therapies, whereas durable remissions are difficult to achieve in patients with tumor, erythrodermic, or systemic disease. We describe a patient with cutaneous T-cell lymphoma and malignant cells constituting 99% of her peripheral blood lymphocytes who had a sustained complete response after an HLA-matched sibling allogeneic stem cell transplantation. We also review the current literature regarding both autologous and allogeneic stem cell transplantations for advanced stages of cutaneous T-cell lymphoma, discuss the importance of the graft-versus-tumor immunomodulatory effect in successful transplantations, and suggest that allogeneic stem cell transplantation deserves further consideration and study as a potential treatment for selected patients who are younger and at high risk.

Total skin electron beam therapy may be associated with improvement of peripheral blood disease in Sézary syndrome.

Total skin electron beam radiation is an effective therapy for palliation of the cutaneous symptoms of the most common types of cutaneous T-cell lymphomas, mycosis fungoides and Sézary syndrome. We report 4 cases of patients with Sézary syndrome who had significant improvement in their blood burden of malignant cells in addition to complete cutaneous responses to total skin electron beam therapy. The data from these 4 patients illustrate the potential for total skin electron beam to be used as both a skin and blood tumor debulking agent, and not merely as a palliation for skin symptoms.