A Young Boy With Hemophagocytic Lymphohistiocytosis Presenting With Vaccine-Related Granulomatous Dermatitis: A Case Report and Literature Review.

ABSTRACT: Cutaneous eruptions associated with hemophagocytic lymphohistiocytosis (HLH) have been reported in 6%-63% of patients. Clinical findings of these skin lesions vary widely and include maculopapular rashes, ulcers, and violaceous nodules. Corresponding histologic findings are also variable and are considered nonspecific. We report the case of a 4-year-old boy who initially developed a widespread popular-pustular rash 2 weeks after his 12-month measles, mumps, and rubella vaccinations. These resolved with scarring then recurred following his 24-month vaccinations. Multiple skin biopsies were negative for infectious organisms and showed a granulomatous infiltrate with perforation and necrobiosis. The differential diagnosis included perforating granuloma annulare, infection, or rheumatoid nodules. At the age of 4, he developed fever, hepatosplenomegaly, pancytopenia and other laboratory abnormalities, requiring hospitalization. A number of studies were performed including biopsies of bone marrow and liver. Molecular testing revealed 2 mutations in UNC13D known to be associated with familial HLH. His prior cutaneous lesions were likely caused by immune dysregulation exacerbated by immunizations because of underlying familial HLH. This case illustrates the importance of recognizing an unusual cutaneous manifestation of a rare disease to arrive at an earlier diagnosis in a pediatric patient. Although cutaneous eruptions usually develop concurrently with other systemic symptoms of HLH, preceding unusual skin lesions may be the first indication of this rare disease.

Health providers induced iatrogenic delusions of infestation.

Patients that suffer from factitial dermatosis mutilate their skin, often lacking any consciousness of self-injury, attributing the resulting lesions to spontaneous development. The case hereby described shows how the health providers' interventions led a patient from a baseline undiagnosed factitious disorder to frank delusions of infestation with Mycobacterium Kansasii, and a relentless search for antibiotic treatments. We highlight the need for educating health practitioners on the characteristics of psycho-cutaneous disorders.

Laugier-Hunziker syndrome: a case of asymptomatic mucosal and acral hyperpigmentation.

Laugier-Hunziker syndrome (LHS) is a rare condition characterized by acquired hyperpigmentation involving the lips, oral mucosa, acral surfaces, nails and perineum. While patients with LHS may manifest pigmentation in all of the aforementioned areas, most present with pigmentation localized to only a few of these anatomical sites. We herein report a patient exhibiting the characteristic pigment distribution pattern associated with LHS. Since LHS is a diagnosis based on exclusion, we discuss the differential diagnosis of mucocutaneous hyperpigmentation. Due to the benign nature of the disease, it is critical to differentiate this disorder from conditions with similar mucocutaneous pigmentary changes with somatic abnormalities that require medical management. We also explore potential mechanisms that may explain the pathogenesis of LHS.

Clinical resolution of pemphigus vulgaris on rituximab.

Although significant progress has been made for the treatment of pemphigus vulgaris (PV) with rituximab (RTX), a consensus remains to be determined for standard treatment protocol, regarding optimal dosing, infusion regimen, and use of concomitant immunotherapy to achieve safe, effective, and rapid clinical response. We describe a patient with pemphigus vulgaris treated with high dose rituximab with the rheumatoid arthritis protocol along with intravenous immunoglobulin therapy. This case provides evidence towards the growing body of research needed to modify and improve treatment for pemphigus using rituximab.

The Relationship Between High Pulse Pressure and Low Ankle-Brachial Index. Potential Utility in Screening for Peripheral Artery Disease in Population-Based Studies.

INTRODUCTION: The ankle-brachial index (ABI) is a reliable screening procedure for peripheral artery disease detection. However, ABI testing is time-consuming and requires trained personnel, which may preclude its routine use in population-based surveys. Preliminary data suggest a relationship between ABI values and pulse pressure (PP) levels. AIM: To assess whether PP calculation might help to detect persons who need ABI screening in population-based studies. METHODS: All Atahualpa residents aged ≥60 years were identified during a door-to-door survey and invited to undergo ABI testing. Non-consented persons and those with ABI ≥1.4 were excluded. Using generalized linear and logistic regression models adjusted for demographics and cardiovascular risk factors, as well as receiver operator characteristics curve analysis, we evaluated the association between PP values and ABI, as well as the reliability of PP to identify candidates for ABI testing. RESULTS: Out of 239 participants (mean age 70 ± 8 years, 62 % women), 46 (19 %) had an ABI ≤0.9 and 136 (57 %) had PP >65 mmHg, with a negative relationship between them (R = -0.386, p < 0.0001). A PP >65 mmHg was associated with an ABI ≤ 0.9 in the logistic regression model (OR 3.46, 95 % CI 1.07-11.2, p = 0.038). Continuous PP levels also correlated negatively with ABI (ß -0.0014, 95 % CI -0.0024 to -0.0004, p = 0.005). The sensitivity of a PP >65 mmHg to predict a low ABI was 85 %, and the specificity was 50 %. In contrast, the sensitivity of blood pressure ≥140/90 mmHg was 27 % and the specificity was 10 %. The area under the curve for the predictive value of a PP >65 mmHg was 0.673 (95 % CI 0.609-0.736), and that of a blood pressure ≥140/90 mmHg was 0.371 (95 % CI 0.30-0.443), with a significant difference between them (p < 0.0001). CONCLUSIONS: PP calculation may be a simple tool to detect candidates for ABI testing in population-based studies.

The association of ankle-brachial index with silent cerebral small vessel disease: results of the Atahualpa Project.

BACKGROUND: An abnormal ankle-brachial index has been associated with overt stroke and coronary heart disease, but little is known about its relationship with silent cerebral small vessel disease. AIM: To assess the value of ankle-brachial index as a predictor of silent small vessel disease in an Ecuadorian geriatric population. METHODS: Stroke-free Atahualpa residents aged ≥60 years were identified during a door-to-door survey. Ankle-brachial index determinations and brain magnetic resonance imaging were performed in consented persons. Ankle-brachial index ≤0.9 and ≥1.4 were proxies of peripheral artery disease and noncompressible arteries, respectively. Using logistic regression models adjusted for age, gender, and cardiovascular health status, we evaluated the association between abnormal ankle-brachial index with silent lacunar infarcts, white matter hyperintensities, and cerebral microbleeds. RESULTS: Mean age of the 224 participants was 70 ± 8 years, 60% were women, and 80% had poor cardiovascular health status. Ankle-brachial index was ≤0.90 in 37 persons and ≥1.4 in 17. Magnetic resonance imaging showed lacunar infarcts in 27 cases, moderate-to-severe white matter hyperintensities in 47, and cerebral microbleeds in 26. Adjusted models showed association of lacunar infarcts with ankle-brachial index ≤ 0.90 (OR: 3.72, 95% CI: 1.35-10.27, P = 0.01) and with ankle-brachial index ≥ 1.4 (OR: 3·85, 95% CI: 1.06-14.03, P = 0.04). White matter hyperintensities were associated with ankle-brachial index ≤ 0.90 (P = 0.03) and ankle-brachial index ≥ 1.4 (P = 0.02) in univariate analyses. There was no association between ankle-brachial index groups and cerebral microbleeds. CONCLUSIONS: In this population-based study conducted in rural Ecuador, apparently healthy individuals aged ≥60 years with ankle-brachial index values ≤0.90 and ≥1.4 are almost four times more likely to have a silent lacunar infarct. Ankle-brachial index screening might allow recognition of asymptomatic people who need further investigation and preventive therapy.

Prevalence, correlates, and prognosis of peripheral artery disease in rural ecuador-rationale, protocol, and phase I results of a population-based survey: an atahualpa project-ancillary study.

Background. Little is known on the prevalence of peripheral artery disease (PAD) in developing countries. Study design. Population-based study in Atahualpa. In Phase I, the Edinburgh claudication questionnaire (ECQ) was used for detection of suspected symptomatic PAD; persons with a negative ECQ but a pulse pressure ≥65 mmHg were suspected of asymptomatic PAD. In Phase II, the ankle-brachial index will be used to test reliability of screening instruments and to determine PAD prevalence. In Phase III, participants will be followed up to estimate the relevance of PAD as a predictor of vascular outcomes. Results. During Phase I, 665 Atahualpa residents aged ≥40 years were enrolled (mean age: 59.5 ± 12.6 years, 58% women). A poor cardiovascular health status was noticed in 464 (70%) persons of which 27 (4%) had a stroke and 14 (2%) had ischemic heart disease. Forty-four subjects (7%) had suspected symptomatic PAD and 170 (26%) had suspected asymptomatic PAD. Individuals with suspected PAD were older, more often women, and had a worse cardiovascular profile than those with nonsuspected PAD. Conclusions. Prevalence of suspected PAD in this underserved population is high. Subsequent phases of this study will determine whether prompt detection of PAD is useful to reduce the incidence of catastrophic vascular diseases in the region.

Utilization of trained volunteers decreases 30-day readmissions for heart failure.

Background: This study evaluated the effectiveness of using trained volunteer staff in reducing 30-day readmissions of congestive heart failure (CHF) patients.Methods: From June 2010 to December 2010, 137 patients (mean age 73 years) hospitalized for CHF were randomly assigned to either: an interventional arm (arm A) receiving dietary and pharmacologic education by a trained volunteer, follow-up telephone calls within 48 hours, and a month of weekly calls; ora control arm (arm B) receiving standard care. Primary outcomes were 30-day readmission rates for CHF and worsening New York Heart Association (NYHA) functional classification; composite and all-cause mortality were secondary outcomes.Results: Arm A patients had decreased 30-day readmissions (7% vs 19%; P ! .05) with a relative risk reduction (RRR) of 63% and an absolute risk reduction (ARR) of 12%. The composite outcome of 30-day readmission, worsening NYHA functional class, and death was decreased in the arm A (24% vs 49%;P ! .05; RRR 51%, ARR 25%). Standard-care treatment and hypertension, age $65 years and hypertension,and cigarette smoking were predictors of increased risk for readmissions, worsening NYHA functional class, and all-cause mortality, respectively, in the multivariable analysis.Conclusions: Utilizing trained volunteer staff to improve patient education and engagement might be an efficient and low-cost intervention to reduce CHF readmissions.

Utilization of trained volunteers decreases 30-day readmissions for heart failure.

BACKGROUND: This study evaluated the effectiveness of using trained volunteer staff in reducing 30-day readmissions of congestive heart failure (CHF) patients. METHODS: From June 2010 to December 2010, 137 patients (mean age 73 years) hospitalized for CHF were randomly assigned to either: an interventional arm (arm A) receiving dietary and pharmacologic education by a trained volunteer, follow-up telephone calls within 48 hours, and a month of weekly calls; or a control arm (arm B) receiving standard care. Primary outcomes were 30-day readmission rates for CHF and worsening New York Heart Association (NYHA) functional classification; composite and all-cause mortality were secondary outcomes. RESULTS: Arm A patients had decreased 30-day readmissions (7% vs 19%; P < .05) with a relative risk reduction (RRR) of 63% and an absolute risk reduction (ARR) of 12%. The composite outcome of 30-day readmission, worsening NYHA functional class, and death was decreased in the arm A (24% vs 49%; P < .05; RRR 51%, ARR 25%). Standard-care treatment and hypertension, age ≥65 years and hypertension, and cigarette smoking were predictors of increased risk for readmissions, worsening NYHA functional class, and all-cause mortality, respectively, in the multivariable analysis. CONCLUSIONS: Utilizing trained volunteer staff to improve patient education and engagement might be an efficient and low-cost intervention to reduce CHF readmissions.