Cytokines and Effector/Regulatory Cells Characterization in the Physiopathology of Cutaneous Lupus Erythematous: A Cross-Sectional Study.

We compared the presence of diverse cytokines and regulatory T and B cells in skin biopsies of discoid lupus erythematosus (DLE) and subacute cutaneous lupus erythematosus (SCLE). We included 19 patients with DLE, 13 with SCLE, 8 healthy controls, and 5 patients with hypertrophic scars. We assessed the CLASI activity score. To determine IL-22-producing cells and the subpopulation of CD4(+)/IL-17A(+)-, CD4(+)/IL-4(+)-, and CD4(+)/IFN-γ (+)-expressing T cells, CD123(+)/IDO(+) pDCs, CD25(+)/Foxp3(+) Tregs, and CD20(+)/IL-10(+)-producing B cells, an immunostaining procedure was performed. Also intracellular IL-22, IL-17, IL-4, IFN-γ, and Foxp3 in CD4 T cells, IL-10 in B cells, and IDO in pDCs were analyzed by flow cytometry in peripheral blood. The main cellular participation in both lupus groups was IL-17- and IL-22-producing cell responses both at skin and at peripheral blood but prevailed in DLE. The CLASI activity scores negatively correlated with Th22 subpopulation and positively correlated with CD25(+)/Foxp3(+) Treg cells. In conclusion a proinflammatory and regulatory imbalance coexists in cutaneous lupus, both responses being more intense in DLE.

Acrometastasis: The Tip of the Iceberg of Metastatic Disease from Thyroid Cancer. Two Cases Report.

Acrometastasis, especially in the hands and fingers, is a rare clinical condition resulting from primary cancers such as lung, breast, kidney, and, rarely, thyroid cancer. Acrometastasis tends to be the tip of the iceberg in patients with extensive systemic disease, which could be regional, pulmonary, skeletal, neurological, or all of them combined. Even though these tumors are clearly visible and symptomatic, the diagnosis is usually misleading because such distal metastatic disease is not thought of at first. In general, systemic treatments should be given to any patient presenting digital acrometastasis. We describe two cases of papillary thyroid carcinoma and digital acrometastasis as a sign of advanced disease.

Emphysematous Pyelonephritis and Emphysematous Cholecystitis: A Result of Uncontrolled Type 2 Diabetes.

Background: Emphysematous pyelonephritis (EPN) is a life-threatening necrotizing infection that results in the presence of gas in the renal parenchyma, collecting system, and surrounding tissues. Up to 95% of patients with EPN have underlying uncontrolled diabetes mellitus. Emphysematous cholecystitis (EC) is a necrotizing infection defined by the presence of gas in the gallbladder. Concurrent presentation of EPN and EC is limited to anecdotal cases in the literature. Case Report: We present the case of a 44-year-old female who arrived at the emergency department with signs of septic shock and diffuse abdominal pain. Diagnosis of EPN and EC was confirmed. Because the patient did not improve after aggressive medical therapy and percutaneous drainage and cholecystostomy, she was taken to surgery for emergency nephrectomy and cholecystectomy. Conclusion: In unusually extensive and severe cases of EPN, medical and minimally invasive procedures are not enough to control the infection. More aggressive management, including emergency surgery, should be implemented in selected patients who present with refractory septic shock associated with extensive disease.

Hepatic epithelioid hemangioendothelioma: a diagnostic challenge of vascular tumors.

Epitheloid hemangioendothelioma is a very rare tumor, with a variable presentation and unpredictable clinical behavior. The etiology and the triggering risk factors have not been specified. Unlike other primary liver tumors, it does not arise in the background of chronic liver disease. The approach is challenging due to the spectrum of possibilities and the need for immunohistochemistry to establish the definitive diagnosis. The information available so far is limited due to the few published cases, this favors that the therapeutic options are few or that there is insufficient evidence to standardize them when the lesion is not resectable.

El hemangioendotelioma epiteloide es un tumor muy infrecuente, con una presentación variable y un comportamiento clínico impredecible. No se han precisado la etiología ni los factores de riesgo desencadenantes. A diferencia de otros tumores hepáticos primarios, no surge en el contexto de una enfermedad crónica del hígado. El abordaje es un reto debido al espectro de posibilidades y la necesidad de inmunohistoquímica para establecer el diagnóstico definitivo. La información disponible hasta el momento es limitada por los escasos casos publicados, lo que favorece que las opciones terapéuticas sean pocas o no exista la evidencia suficiente para estandarizarlas cuando la lesión no sea resecable.

Fibromatosis gástrica: un tumor raro en una localización infrecuente. Reporte de un caso.

La fibromatosis tipo desmoide es un tumor benigno de suma rareza con características localmente agresivas. Se desarrolla en especial en tejidos blandos por su origen en los músculos y las aponeurosis. El diagnóstico se establece por inmunohistoquímica con positividad para vimentina, B-catenina y en ocasiones para actina de músculo liso. El espectro clínico de presentación es amplio, y según este y la resecabilidad se ofrece el tratamiento. Presentamos el caso de una paciente con síntomas de obstrucción al vaciamiento gástrico secundaria a una lesión subepitelial en cuerpo con inmunohistoquímica concluyente para fibromatosis gástrica.Desmoid-type fibromatosis is an extremely rare benign tumor with locally aggressive features. It is predominantly developing in soft tissues due to its origin in muscles and aponeurosis. The diagnosis is established by immunohistochemistry with positivity for vimentin, B-catenin and sometimes for smooth muscle actin. The clinical spectrum of presentation is wide, based on this and resectability the treatment is offered. We present the case of a patient with symptoms of gastric outlet obstruction secondary to a subepithelial lesion in the gastric body with conclusive immunohistochemistry for gastric fibromatosis.

Elastographic ultrasound: an additional image tool in Sjögren's syndrome.

AIM: To evaluate the stiffness of parotid and submandibular glands using elastography ultrasound and to correlate it with B-mode ultrasonographical, clinical and serological features, salivary profibrotic and inflammatory chemokines, and salivary gland fibrosis. METHODS: We performed B-mode and elastography ultrasound of major salivary glands of 26 patients with primary Sjögren's syndrome. We registered the shear wave velocity (SWV) and correlated it with the morphologic ultrasonographic changes assessed by the Hocevar scale. We assessed the European League Against Rheumatism (EULAR) Sjögren's Syndrome Disease Activity Index (ESSDAI), EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI), non-stimulated whole salivary flow rate (NSWSF), C3 and C4 levels, anti-Ro/La antibodies, salivary inflammatory (C-X-C motif ligand 13 [CXCL13], CXCL10, CXCL8, C-C motif ligand 2 [CCL2], interleukin 10 [IL-10] and IL-6) and pro-fibrotic (CXCL14, CCL28, tumor necrosis factor-related apoptosis-inducing ligand and transforming growth factor ß) chemokines and cytokines and evaluated the presence of fibrosis in the minor salivary gland. RESULTS: Ninety-two percent of patients were women; mean age was 51.1 ± 11 years; median disease duration was 6.1 years; 92.3% had oral symptoms and 26.9% fibrosis. The median B-mode score was 22.2 points and the median SWV 2.5 m/s (τ = 0.53, P = 0.001). The SWV correlated with the NSWSF (τ = -0.53, P = 0.001), ESSDAI (τ = 0.31, P = 0.03), glandular ESDDAI domain (τ = 0.36, P = 0.02), C4 levels (τ = -0.32, P = 0.04), salivary CXCL13 (τ = 0.29, P = 0.03) and CXCL10 (τ = 0.30, P = 0.003), but not with age and fibrosis. CONCLUSION: WV correlated with the B-mode ultrasound score, systemic and glandular activity and in a large degree with CXCL10, an inflammatory chemokine, but not with fibrosis. An increased SWV might represent chronic glandular inflammation rather than fibrotic changes in these patients.

Quality of life in patients with antiphospholipid syndrome is related to disease burden and anticoagulant therapy.

AIM: To evaluate health-related quality of life (HRQoL) in primary antiphospholipid syndrome (PAPS) and correlate it with a crude estimate of accrual organ damage, comorbidity (diabetes mellitus, hypertension and dyslipidemia) and treatment (oral anticoagulation, immunosuppressors and prednisone). METHODS: We assessed HRQoL with the Short-Form 36 (SF-36) and the Lupus Quality of Life instrument (LupusQoL) and the disease burden with a modified Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR SDI). As controls we used SF-36 data from a Mexican general population within the same age range. RESULTS: We included 50 PAPS patients (86% women), mean age 47.6 ± 14.5 years, median disease duration 9.4 years, median SLICC/ACR score of 1 point and 80% had thrombotic events. PAPS patients had lower HRQoL than controls. We found a positive correlation between SF-36 and LupusQoL (r = 0.85, P < 0.0001). The SLICC/ACR SDI correlated negatively with both LupusQoL and SF-36, specifically the peripheral vascular domain (r = -0.29, P = 0.03, for both). Patients on oral anticoagulants (n = 37) had lower LupusQoL, physical functioning, intimate relationships, burden to others and pain scores as well as a lower SF-36 physical functioning score. We did not find differences in HRQoL regarding comorbidities and other treatments. CONCLUSIONS: HRQoL in PAPS was related to burden of the disease specifically at the vascular peripheral area and use of anticoagulants.

Virus del papiloma humano en carcinoma colorrectal / Human papilloma virus in colorectal carcinoma

Resumen Introducción: Previos trabajos han reportado una asociación entre la infección del virus del papiloma humano (VPH) y el desarrollo de cáncer colorrectal, aunque existe controversia al respecto. Materiales y Método: Estudio observacional, transversal, descriptivo, retrospectivo, no ciego. Se utilizaron 50 muestras de patología con diagnóstico de adenocarcinoma colorrectal, incluidas en parafina, para aislar ADN de las muestras. Se realizó la extracción de ADN mediante protocolos establecidos para extracción, lisis y rehidratación de muestra. Se identificó y genotipicó el ADN del virus para amplificar y detectar subtipos oncogénicos de entre 35 subtipos diferentes incluidos en la prueba, secuenciando las muestras positivas, utilizando protocolos ya establecidos de purificación y análisis de muestra, mediante microarreglos. Resultados: Se identificaron 14 muestras de 50 (28%) estudiadas positivas para el virus de papiloma humano de las cuales 11 (22%) incluyen uno o más subtipos de alto riesgo para neoplasia. No se identificaron diferencias estadísticamente significativas entre grupos en cuanto a edad, sexo, localización del tumor, grado de diferenciación, infiltración, ganglios afectados, metástasis o número de paquetes/año. Conclusión: La detección de los subtipos de VPH de alto riesgo en un alto porcentaje de las muestras positivas, sugiere una asociación entre la infección con el desarrollo de cáncer colorrectal.

Introduction: Previous works have reported an association between human papilloma virus (HPV) infection and the development of colorectal cancer, and although controversy regarding this association exists. Materials and Method: This was an observational, cross-sectional, descriptive, retrospective unblinded study. Fifty pathology samples embedded in paraffin with a diagnosis of colorectal adenocarcinoma were used to isolate DNA from the tissue. DNA was extracted according to established protocols for extraction, lysis and sample rehydration. Viral DNA was identified and genotypified to amplify and detect oncogenic subtypes among 35 different subtypes included in the study, sequencing positive samples with established protocols of purification and sample analysis using microarrays. Results: Fourteen of 50 (28%) samples were identified as positive for human papilloma virus; of these 11 (22%) included one or more high-risk subtypes for neoplasia. Statistically significant differences were not found between the groups regarding age, sex, tumor location, degree of differentiation, infiltration, affected lymph nodes, metastasis and number of pack years. Conclusion: The detection of high-risk VPH subtypes in a high percentage of positive samples, suggests an association between infection and the development of colorectal cancer.

Manejo de la ruptura hepática espontánea en el síndrome de HELLP / Management of spontaneous hepatic rupture in HELLP syndrome

Introducción. El síndrome de HELLP es una variante grave de la preeclampsia, con una baja incidencia, entre el 0,5-0,9 % de todos los embarazos. La ruptura hepática espontánea en el embarazo es infrecuente, presenta una alta mortalidad, y se ha relacionado con hemangiomas hepáticos, coriocarcinoma y síndrome de HELLP. Caso clínico. Mujer de 29 años con embarazo de 34,3 semanas, con actividad uterina progresiva, sufrimiento fetal agudo y hallazgos compatibles con síndrome de HELLP. Se practicó cesárea de urgencia encontrando hemoperitoneo y ruptura hepática del lóbulo derecho, por lo que se realizó empaquetamiento abdominal y posterior reintervención quirúrgica. Discusión. La ruptura hepática espontánea en el embarazo es una entidad infrecuente y potencialmente mortal. Es esencial un alto nivel de sospecha para realizar el diagnóstico y la rápida intervención. Requiere un manejo multidisciplinario para un resultado exitoso. Se han descrito múltiples tratamientos que dependen de las manifestaciones clínicas y extensión de la lesión, pero es claro que la laparotomía primaria y el taponamiento constituyen la mejor elección ante el hallazgo intraoperatorio.

Introduction. HELLP syndrome is a severe variant of preeclampsia with a low incidence, between 0.5-0.9% of all pregnancies. Spontaneous hepatic rupture in pregnancy is rare, has a high mortality, and has been associated with hepatic hemangiomas, choriocarcinoma, and HELLP syndrome. Clinical case. 29-year-old woman with a 34.3-week pregnancy, with progressive uterine activity and acute fetal distress and findings compatible with HELLP syndrome. An emergency cesarean section was performed, finding hemoperitoneum and hepatic rupture of the right lobe, performing abdominal packing and subsequent surgical reoperation. Discussion. Spontaneous liver rupture in pregnancy is a rare and potentially fatal entity. A high level of suspicion is essential to make the diagnosis and prompt intervention. It requires multidisciplinary management for a successful outcome. Multiple treatments have been described that depend on the clinical manifestations and extent of the lesion, but it is clear that primary laparotomy and packing constitute the best choice before the intraoperative finding.

Manejode linfoma primario de bazo en paciente con ViH-Sida: reporte de caso / Management of primary lymphoma of the spleen in a patient with HIV-AIDS: case report

Los linfomas primarios de bazo afectan al bazo y/o sus ganglios linfáticos inicialmente. Representan <1% de los linfomas e implican un reto diagnóstico, por baja prevalencia y sintomatología inusual. Por lo tanto, el objetivo de este trabajo fue describir el manejo y evolución de un paciente con VIH-SIDA y la presentación de un Linfoma primario de bazo.En este reporte presentamos el caso de un paciente masculino de 52 años con VIH sin apego a tratamiento debuta con dolor abdominal, fiebre y pérdida de peso de 2 meses de evolución. La tomografía computarizada properatoria revela esplenomegalia y lesiones hipodensas sospechosas de neoformación. Es intervenido, realizándose una laparotomía exploratoria, con esplenectomía. El estudio histopatológico reporta linfoma no Hodgkin.El linfoma primario de bazo es poco frecuente. Es necesario su conocimiento para su sospecha diagnóstica y un diagnóstico diferencial en pacientes con esplenomegalia.Primary splenic lymphomas involve the spleen and/or its lymph nodes. They represent <1% of lymphomas and they imply a diagnostic challenge due to its poor symptomatology and low prevalence.There for, the objects this work describe the management and evolution of a patient with HIV-AIDS and presentation of a primary lymphoma of the spleenIn this report we present the case of a 52-year-old male patient with HIV infection without treatment presented with abdominal pain, fever and a 2-month weight loss. The pre-operative computed tomography showed splenomegaly and hypodense lesions suspected to be tumoral. He was operated on, performing an exploratory laparotomy, performing an splenectomy. The histopathological study reported a non-Hodgkin lymphoma.Primary splenic lymphoma is a non frequent entity. It is necessary to be acquainted with it in order to suspect it.

Primary splenic lymphomas involve the spleen and/or its lymph nodes. They represent <1% of lymphomas and they imply a diagnostic challenge due to its poor symptomatology and low prevalence.There for, the objects this work describe the management and evolution of a patient with HIV-AIDS and presentation of a primary lymphoma of the spleenIn this report we present the case of a 52-year-old male patient with HIV infection without treatment presented with abdominal pain, fever and a 2-month weight loss. The pre-operative computed tomography showed splenomegaly and hypodense lesions suspected to be tumoral. He was operated on, performing an exploratory laparotomy, performing an splenectomy. The histopathological study reported a non-Hodgkin lymphoma.Primary splenic lymphoma is a non frequent entity. It is necessary to be acquainted with it in order to suspect it.