Suppression of age-related salivary gland autoimmunity by glycosylation-dependent galectin-1-driven immune inhibitory circuits.

Aging elicits quantitative and qualitative changes in different immune components, leading to disruption of tolerogenic circuits and development of autoimmune disorders. Galectin-1 (Gal1), an endogenous glycan-binding protein, has emerged as a regulator of immune cell homeostasis by shaping the fate of myeloid and lymphoid cells. Here, we demonstrate that aged Gal1-null mutant (Lgals1-/- ) mice develop a spontaneous inflammatory process in salivary glands that resembles Sjögren's syndrome. This spontaneous autoimmune phenotype was recapitulated in mice lacking ß1,6N-acetylglucosaminyltransferase V (Mgat5), an enzyme responsible for generating ß1,6-branched complex N-glycans, which serve as a major ligand for this lectin. Lack of Gal1 resulted in CD11c+ dendritic cells (DCs) with higher immunogenic potential, lower frequency of Foxp3+ regulatory T cells (Tregs), and increased number of CD8+ T cells with greater effector capacity. Supporting its tolerogenic activity, Gal1 expression decreased with age in autoimmunity-prone nonobese diabetic (NOD) mice. Treatment with recombinant Gal1 restored tolerogenic mechanisms and reduced salivary gland inflammation. Accordingly, labial biopsies from primary Sjögren's syndrome patients showed reduced Gal1 expression concomitant with higher number of infiltrating CD8+ T cells. Thus, endogenous Gal1 serves as a homeostatic rheostat that safeguards immune tolerance and prevents age-dependent development of spontaneous autoimmunity.

[Brunner's gland adenoma: a rare tumor as cause of pyloric syndrome in an AIDS patient]. / Adenoma de glándulas de Brunner: un tumor raro como causa de sindrome pilórico en un paciente con SIDA.

Brunner's gland adenoma is a rare neoplasm that accounts for only the 0.008% of all benign duodenal tumors. Here we describe the case ofan HIV-seropositive man who developed a severe pyloric stenosis due to a Brunner's adenoma of the bulb and the first duodenal portion. Gastroduodenoscopy showed a large polypoid tumor that obstructed the pyloric region. The lesion was resected by surgery and a gastroduodenal anastomosis was made. The histopathologic examination of the surgical specimen showed a large proliferation of Brunner's glands into a large pedunculated polyp that confirmed the diagnosis of this hamartoma.

Progesterone receptor assembly of a transcriptional complex along with activator protein 1, signal transducer and activator of transcription 3 and ErbB-2 governs breast cancer growth and predicts response to endocrine therapy.

INTRODUCTION: The role of the progesterone receptor (PR) in breast cancer remains a major clinical challenge. Although PR induces mammary tumor growth, its presence in breast tumors is a marker of good prognosis. We investigated coordinated PR rapid and nonclassical transcriptional effects governing breast cancer growth and endocrine therapy resistance. METHODS: We used breast cancer cell lines expressing wild-type and mutant PRs, cells sensitive and resistant to endocrine therapy, a variety of molecular and cellular biology approaches, in vitro proliferation studies and preclinical models to explore PR regulation of cyclin D1 expression, tumor growth, and response to endocrine therapy. We investigated the clinical significance of activator protein 1 (AP-1) and PR interaction in a cohort of 99 PR-positive breast tumors by an immunofluorescence protocol we developed. The prognostic value of AP-1/PR nuclear colocalization in overall survival (OS) was evaluated using Kaplan-Meier method, and Cox model was used to explore said colocalization as an independent prognostic factor for OS. RESULTS: We demonstrated that at the cyclin D1 promoter and through coordinated rapid and transcriptional effects, progestin induces the assembly of a transcriptional complex among AP-1, Stat3, PR, and ErbB-2 which functions as an enhanceosome to drive breast cancer growth. Our studies in a cohort of human breast tumors identified PR and AP-1 nuclear interaction as a marker of good prognosis and better OS in patients treated with tamoxifen (Tam), an anti-estrogen receptor therapy. Rationale for this finding was provided by our demonstration that Tam inhibits rapid and genomic PR effects, rendering breast cancer cells sensitive to its antiproliferative effects. CONCLUSIONS: We here provided novel insight into the paradox of PR action as well as new tools to identify the subgroup of ER+/PR + patients unlikely to respond to ER-targeted therapies.

[Penile nodules, a rare clinical manifestation of lymphogranuloma venereum]. / Nódulos peneanos, una rara manifestación clínica de linfogranuloma venéreo.

Lymphogranuloma venereum is a sexually transmitted disease caused by L1, L2 and L3 serovars of Chlamydia trachomatis. A rare clinical presentation of the primary stage is lymphangitis of the penis, with the appearance of one or more tense lymph nodes classically called "buboes". We report the case of an HIV-positive patient with sexually transmitted disease risk behaviors with penile nodules as a manifestation of lymphogranuloma venereum.

El linfogranuloma venéreo es una infección de transmisión sexual (ITS) causada por las serovariedades L1, L2 y L3 de Chlamydia trachomatis. Una forma rara de presentación del estadio primario es la linfangitis localizada en pene, con la formación de uno o más nódulos linfáticos tensos clásicamente llamados "bubonódulos" (bubón pequeño). Presentamos el caso de un paciente HIV positivo con conductas de riesgo para ITS con nódulos peneanos como manifestación de linfogranuloma venéreo.

Clinical relevance of ErbB-2/HER2 nuclear expression in breast cancer.

BACKGROUND: The biological relevance of nuclear ErbB-2/HER2 (NuclErbB-2) presence in breast tumors remains unexplored. In this study we assessed the clinical significance of ErbB-2 nuclear localization in primary invasive breast cancer. The reporting recommendations for tumor marker prognostic studies (REMARK) guidelines were used as reference. METHODS: Tissue microarrays from a cohort of 273 primary invasive breast carcinomas from women living in Chile, a Latin American country, were examined for membrane (MembErbB-2) and NuclErbB-2 expression by an immunofluorescence (IF) protocol we developed. ErbB-2 expression was also evaluated by immunohistochemistry (IHC) with a series of antibodies. Correlation between NuclErbB-2 and MembErbB-2, and between NuclErbB-2 and clinicopathological characteristics of tumors was studied. The prognostic value of NuclErbB-2 in overall survival (OS) was evaluated using Kaplan-Meier method, and Cox model was used to explore NuclErbB-2 as independent prognostic factor for OS. RESULTS: The IF protocol we developed showed significantly higher sensitivity for detection of NuclErbB-2 than IHC procedures, while its specificity and sensitivity to detect MembErbB-2 were comparable to those of IHC procedures. We found 33.6% NuclErbB-2 positivity, 14.2% MembErbB-2 overexpression by IF, and 13.0% MembErbB-2 prevalence by IHC in our cohort. We identified NuclErbB-2 positivity as a significant independent predictor of worse OS in patients with MembErbB-2 overexpression. NuclErbB-2 was also a biomarker of lower OS in tumors that overexpress MembErbB-2 and lack steroid hormone receptors. CONCLUSIONS: We revealed a novel role for NuclErbB-2 as an independent prognostic factor of poor clinical outcome in MembErbB-2-positive breast tumors. Our work indicates that patients presenting NuclErbB-2 may need new therapeutic strategies involving specific blockage of ErbB-2 nuclear migration.

[Malignant syphilis in a patient infected by human immunodeficiency virus. Case report and literature review]. / Sífilis maligna en un paciente con infección por VIH: Presentación de un caso y revisión de la literatura.

Malignant syphilis is a rare form of secondary syphilis strongly associated with human immunodeficiency virus infection (HIV). This clinical form of the disease is characterized by atypical cutaneous ulcerative and disseminated lesions and systemic compromise that can delay the final diagnosis. There are only few reports in the medical literature about malignant lues in HIV-infected patients. Malignant syphilis should be considered in the differential diagnosis in HIV-infected patients with fever and ulcerative skin lesions. Here we describe a man who developed clinical cutaneous and systemic manifestations pathologically confirmed as malignant syphilis and we performed a review of the literature.

[Skin manifestations in patients with COVID-19. A prospective study]. / Manifestaciones cutáneas en pacientes con COVID-19. Estudio prospectivo.

As of March 2020, skin lesions associated with COVID-19 have been described. The objectives of the study were to characterize the skin lesions in these patients, analyze their temporal relationship, association with the severity of the disease, extracutaneous symptoms and laboratory parameters. A prospective, observational, analytical and cross-sectional study was conducted in hospitalized patients diagnosed with COVID-19. Dermatoses were classified as primary and secondary. Forty-five patients were included, 44.4% with primary dermatoses and 53.3% with secondary lesions. The mean age was 46 years (SD: 17), with a male predominance (68.9%). The primary lesions appeared after a median of 5 days (IQR: 3-10) from the onset of COVID-19 symptoms and the secondary ones after 14.5 days (IQR: 7-20). The primary dermatoses found were maculopapular rash (65%), urticarial (20%, half with vesicular lesions), livedo reticular (10%) and purpura (5%). The most frequent secondary dermatoses were adverse drug reactions (37.1%) and infectious dermatoses (25.9%). Maculopapular rash was associated with moderate COVID-19 and pressure injuries with severe COVID-19 (p < 0.05). The finding of neutrophilia was higher among those with secondary infectious dermatoses (p < 0.05). No significant differences were found when evaluating other laboratory parameters. This work shows the skin manifestations in patients hospitalized with COVID-19 in our environment. The most prevalent pattern was the maculopapular rash that was associated with the moderate form of the disease. The appearance of lesions 2 weeks after the onset of COVID-19 symptoms was associated with secondary dermatoses.

Desde marzo 2020 se describieron lesiones cutáneas asociadas a COVID-19. Los objetivos del estudio fueron caracterizar las lesiones cutáneas en estos pacientes, analizar su relación temporal, asociación con la gravedad de la enfermedad, los síntomas extracutáneos y parámetros de laboratorio. Es un estudio prospectivo, observacional, analítico y de corte transversal, en internados con diagnóstico de COVID-19. Se catalogaron las dermatosis en primarias y secundarias. Se incluyeron 45 pacientes, 44.4% con dermatosis primarias y 53.3% con lesiones secundarias. La edad media fue de 46 años (DS: 17), con predominio del sexo masculino (68.9%). Las lesiones primarias aparecieron luego de una mediana de 5 días (RIC: 3-10) del inicio de los síntomas de COVID-19 y las secundarias luego de 14.5 días (RIC: 7-20). Las dermatosis primarias fueron: exantema maculopapuloso (65%), urticariforme (20%, la mitad con lesiones vesiculosas), livedo reticular (10%) y púrpura (5%). Las dermatosis secundarias más frecuentes fueron reacciones adversas a fármacos (37.1%) y dermatosis infecciosas (25.9%). El exantema maculopapuloso se asoció a COVID-19 moderado y las lesiones por presión a COVID-19 grave (p < 0.05). El hallazgo de neutrofilia fue mayor entre aquellos con dermatosis infecciosas secundarias (p < 0.05). No se encontraron diferencias significativas al evaluar otros parámetros de laboratorio, ni síntomas extracutáneos. Este trabajo muestra las manifestaciones cutáneas en internados con COVID-19. El patrón más prevalente fue el exantema maculopapuloso que se asoció con la forma moderada de la enfermedad. La aparición de lesiones luego de las 2 semanas del inicio de los síntomas de COVID-19 se asoció a dermatosis secundarias.

Cromoblastomicosis: abordaje con terapia combinada / Chromoblastomycosis: a combination therapy approach

La cromoblastomicosis es una micosis de implantación crónica y progresiva causada por diversos hongos de la familia Dematiaceae. En Latinoamérica, las especies en-contradas con más frecuencia son Fonsecaea pedrosoi y Cladophialophora carrionii. El tratamiento de esta micosis puede ser un desafío por la falta de respuesta y la recidiva, en especial en individuos con lesiones crónicas y extensas.Se presenta un individuo con recaída de cromoblastomico-sis (causada por Fonsecaea pedrosoi) en miembro inferior derecho que había realizado tratamiento incompleto con terbinafina e itraconazol. El paciente respondió de mane-ra favorable al retratamiento con itraconazol y terbinafina combinado con resección quirúrgica parcial de la lesión e injerto de piel en sitio quirúrgico

Chromoblastomycosis is a chronic and subcutaneous mycosis caused by various dematiaceous fungi, In Latin America, the most frequently found species are Fonsecaea pedrosoi and Cladophialophora carrionii.Treatment is a challenge because of the lack of response and recurrence in in some cases, especially in patients with extensive and chronic lesions.We report an individual with relapse of chromoblastomycosis (by Fonsecaea pedrosoi) in the right lower limb, who had undergone incomplete treatment with terbinafine and itraconazole. The patient responded favorably to retreatment with itraconazole and terbinafine combined with partial surgical resection of the lesion and skin grafting at the surgical site.

Nódulos peneanos, una rara manifestación clínica de linfogranuloma venéreo / Penile nodules, a rare clinical manifestation of lympho granuloma venereum

Resumen El linfogranuloma venéreo es una infección de trans misión sexual (ITS) causada por las serovariedades L1, L2 y L3 de Chlamydia trachomatis. Una forma rara de presen tación del estadio primario es la linfangitis localizada en pene, con la formación de uno o más nódulos linfáticos tensos clásicamente llamados "bubonódulos" (bubón pe queño). Presentamos el caso de un paciente HIV positivo con conductas de riesgo para ITS con nódulos peneanos como manifestación de linfogranuloma venéreo.

Abstract Lymphogranuloma venereum is a sexually trans mitted disease caused by L1, L2 and L3 serovars of Chlamydia trachomatis. A rare clinical presentation of the primary stage is lymphangitis of the penis, with the appearance of one or more tense lymph nodes classically called "buboes". We report the case of an HIV-positive patient with sexually transmitted disease risk behaviors with penile nodules as a manifestation of lymphogranuloma venereum.

Angiomatosis bacilar y sarcoma de Kaposi en lesión cutánea de paciente VIH positivo / Bacillary angiomatosis and Kaposi's sarcoma in skin lesion of HIV positive patient

La angiomatosis bacilar (AB) es una enfermedad infec-ciosa poco frecuente, causada por bacterias del género Bartonella spp. transmitidas por vectores como pulgas, piojos y mosquitos. En el ser humano provoca diferentes síndromes clínicos. En pacientes con infección por el virus de inmunodeficiencia humana (VIH) con recuento de LT CD4 + <100 cél/µL se asocia a lesiones angiomatosas con neovascularización que comprometen la piel y, en menor medida, mucosas, hígado, bazo y huesos.El sarcoma de Kaposi (SK) es una neoplasia caracteriza-da por hiperplasia vascular multifocal de origen endotelial relacionada con el herpes virus humano 8. También puede afectar piel, mucosas y vísceras, siendo la variante epidé-mica una enfermedad marcadora de la infección avanzada por VIH. El principal diagnóstico diferencial clínico para las lesiones cutáneas y mucosas del SK es la AB.Presentamos un paciente con enfermedad VIH/sida que desarrolló AB y SK en forma concomitante en la misma lesión cutánea

Bacillary angiomatosis (BA) is a rare infectious disease, caused by bacteria of the genus Bartonella spp, transmitted by vectors such as fleas, lice and mosquitoes. It causes different clinical syndromes in humans. In patients with human immunodeficiency virus (HIV) infection with an LT CD4 + <100 cell/µL count, it is associated with the development of angiomatous lesions with neovascularization involving the skin and, with less frequency, mucous membranes, liver, spleen and bones. Kaposi's sarcoma (KS) is a neoplasm characterized by multifocal vascular hyperplasia of endothelial origin related to human herpes virus 8. It can also compromiso the skin, mucous membranes and viscera, with the epidemic variant being a marker disease of advanced HIV infection. The main clinical differential diagnosis for KS skin and mucosal lesions is the BA.Herein we present a patient with HIV/AIDS disease that developed BA and KS concomitantly in the same skin lesion

Manifestaciones cutáneas en pacientes con COVID-19. Estudio prospectivo / Skin manifestations in patients with COVID-19. A prospective study

Resumen Desde marzo 2020 se describieron lesiones cutáneas asociadas a COVID-19. Los objetivos del estudio fueron caracterizar las lesiones cutáneas en estos pacientes, analizar su relación temporal, asociación con la gravedad de la enfermedad, los síntomas extracutáneos y parámetros de laboratorio. Es un estudio prospectivo, observacional, analítico y de corte transversal, en internados con diagnóstico de COVID-19. Se catalogaron las dermatosis en primarias y secundarias. Se incluyeron 45 pacientes, 44.4% con dermatosis primarias y 53.3% con lesiones secundarias. La edad media fue de 46 años (DS: 17), con predominio del sexo masculino (68.9%). Las lesiones primarias aparecieron luego de una mediana de 5 días (RIC: 3-10) del inicio de los síntomas de COVID-19 y las secundarias luego de 14.5 días (RIC: 7-20). Las dermatosis primarias fueron: exantema maculopapuloso (65%), urticariforme (20%, la mitad con lesiones vesiculosas), livedo reticular (10%) y púrpura (5%). Las dermatosis secundarias más frecuentes fueron reacciones adversas a fármacos (37.1%) y dermatosis infecciosas (25.9%). El exantema maculopapuloso se asoció a COVID-19 moderado y las lesiones por presión a COVID-19 grave (p < 0.05). El hallazgo de neutrofilia fue mayor entre aquellos con dermatosis infecciosas secundarias (p < 0.05). No se encontraron diferencias significativas al evaluar otros parámetros de laboratorio, ni síntomas extracutáneos. Este trabajo muestra las manifestaciones cutáneas en internados con COVID-19. El patrón más prevalente fue el exantema maculopapuloso que se asoció con la forma moderada de la enfermedad. La aparición de lesiones luego de las 2 semanas del inicio de los síntomas de COVID-19 se asoció a dermatosis secundarias.

Abstract As of March 2020, skin lesions associated with COVID-19 have been described. The objectives of the study were to char acterize the skin lesions in these patients, analyze their temporal relationship, association with the severity of the disease, extracutaneous symptoms and laboratory parameters. A prospective, observational, analytical and cross-sectional study was conducted in hospitalized patients diagnosed with COVID-19. Dermatoses were clas sified as primary and secondary. Forty-five patients were included, 44.4% with primary dermatoses and 53.3% with secondary lesions. The mean age was 46 years (SD: 17), with a male predominance (68.9%). The primary lesions appeared after a median of 5 days (IQR: 3-10) from the onset of COVID-19 symptoms and the secondary ones after 14.5 days (IQR: 7-20). The primary dermatoses found were maculopapular rash (65%), urticarial (20%, half with vesicular lesions), livedo reticular (10%) and purpura (5%). The most frequent secondary dermatoses were adverse drug reactions (37.1%) and infectious dermatoses (25.9%). Maculopapular rash was associated with moderate COVID-19 and pressure injuries with severe COVID-19 (p < 0.05). The finding of neutrophilia was higher among those with secondary infectious dermatoses (p < 0.05). No significant differences were found when evaluating other laboratory parameters. This work shows the skin manifestations in patients hospitalized with COVID-19 in our environment. The most prevalent pattern was the maculopapular rash that was associated with the moderate form of the disease. The appearance of lesions 2 weeks after the onset of COVID-19 symptoms was associated with secondary dermatoses.

Exantema asociado a COVID-19 / COVID-19 skin rash

A partir de marzo de 2020 se han empezado a describir lesiones cutáneas asociadas a COVID-19 que fueron agrupadas en patrones y se relacionaron con la severidad y la temporalidad de la enfermedad. Se presenta el caso de un paciente con COVID-19 leve y lesiones cutáneas que pueden atribuirse a la infección. Se realiza una revisión de las manifestaciones cutáneas asociadas a COVID-19 y la interpretación de los diagnósticos diferenciales que se pensaron en el paciente

Since March 2020, skin lesions associated with COVID-19 have begun to be described. These were grouped into patterns, and were related to the severity and temporality of the disease. A patient with mild COVID-19 and skin lesions that can be attributed to the infection is presented. A review of the cutaneous manifestations associated with COVID-19 and the interpretation of the differential diagnoses that were thought of in the patient is carried out.

A phase II dose-escalation trial of perioperative desmopressin (1-desamino-8-d-arginine vasopressin) in breast cancer patients.

Desmopressin (dDAVP) is a well-known peptide analog of the antidiuretic hormone vasopressin, used to prevent excessive bleeding during surgical procedures. dDAVP increases hemostatic mediators, such as the von Willebrand factor (vWF), recently considered a key element in resistance to metastasis. Studies in mouse models and veterinary trials in dogs with locally-advanced mammary tumors demonstrated that high doses of perioperative dDAVP inhibited lymph node and early blood-borne metastasis and significantly prolonged survival. We conducted a phase II dose-escalation trial in patients with breast cancer, administering a lyophilized formulation of dDAVP by intravenous infusion in saline, 30-60 min before and 24 h after surgical resection. Primary endpoints were safety and tolerability, as well as selection of the best dose for cancer surgery. Secondary endpoints included surgical bleeding, plasma levels of vWF, and circulating tumor cells (CTCs) as measured by quantitative PCR of cytokeratin-19 transcripts. Only 2 of a total of 20 patients experienced reversible adverse events, including hyponatremia (grade 4) and hypersensitivity reaction (grade 2). Reactions were adequately managed by slowing the infusion rate. A reduced intraoperative bleeding was noted with increasing doses of dDAVP. Treatment was associated with higher vWF plasma levels and a postoperative drop in CTC counts. At the highest dose level evaluated (2 µg/kg) dDAVP appeared safe when administered in two slow infusions of 1 µg/kg, before and after surgery. Clinical trials to establish the effectiveness of adjunctive perioperative dDAVP therapy are warranted. This trial is registered on www.clinicaltrials.gov (NCT01606072).

p42/p44 MAPK-mediated Stat3Ser727 phosphorylation is required for progestin-induced full activation of Stat3 and breast cancer growth.

Stat3 is a signaling node for multiple oncogenic pathways and is therefore frequently active in breast cancer. As experimental and clinical evidence reveals that progestins are key players in controlling mammary gland tumorigenesis, we studied Stat3 participation in this event. We have previously shown that progestins induce Stat3Tyr705 phosphorylation and its transcriptional activation in breast cancer cells. In this study, we demonstrate that progestins also induce Stat3 phosphorylation at Ser727 residue, which occurs via activation of c-Src/p42/p44 MAPK pathways in murine progestin-dependent C4HD cells and in T-47D cells. Expression of a Stat3S727A vector, which carries a serine-to-alanine substitution at codon 727, shows that Stat3Ser727 phosphorylation is required for full transcriptional activation of cyclin D1 gene expression by progestins and for in vivo Stat3 recruitment on cyclin D1 promoter. Transfection of Stat3S727A in murine and human breast cancer cells abolished progestin-induced in vitro and in vivo growth. Moreover, we found a positive correlation between progesterone receptor expression and nuclear localization of Stat3Ser727 phosphorylation in breast cancer biopsies. These data highlight Stat3 phosphorylation in Ser727 residue as a nongenomic action by progestins, necessary to promote breast cancer growth.

Identificación de citomegalovirus y varicela zoster virus en lesiones cutáneas en una paciente con sida Presentación de un caso y revisión de la bibliografía / CENTRAL NERVOUS SYSTEM INVOLVEMENT DUE TO HERPESVIRUS REACTIVATION IN AIDS PATIENTS

Las reactivaciones de las infecciones latentes por virus de la familia Herpes originan variadas y graves manifestaciones clínicas en los enfermos con sida. Las lesiones mucocutáneas son comunes en las infecciones por Herpes simple 1 y 2 y por varicela-zóster (VZV). En cambio, son infrecuentes en infecciones por citomegalovirus (CMV). La coexistencia de más de un patógeno en la misma lesión ha sido escasamente referida en la literatura. Presentamos una paciente con enfermedad VIH/sida avanzada que desarrolló lesiones cutáneas diseminadas, en una de las cuales se identificó por técnica de PCR el genoma de VZV y CMV. El diagnóstico precoz seguido del tratamiento antiherpético y la reconstitución inmunológica alcanzada con la TARGA pueden mejorar el pronóstico de esta clase de pacientes

The reactivation of latent infections due to Herpesviridae is associated with a serious compromise in HIV/AIDS patients. Mucocutaneous lesions are frequent in disseminated infections due to Herpes simple 1 and 2 and varicella-zoster virus (VZV). However, cutaneous involvement is rare in cytomegalovirus infections. The coexistence of VZV and CMV in the same lesion has been little reported in the literature. Here, we describe a female with advanced HIV/AIDS disease who developed disseminated cutaneous lesions, in one of yhem we detected VZV and CMV by PCR. Early diagnosis followed by specific antiherpetic therapy and the immune reconstitution associated with HAART can improve the prognosis of these kind of patients.

Elastosis perforante serpiginosa y pseudo-pseudoxantoma elástico secundarias y tratamiento con D- penicilamina en paciente con enfermedad de Wilson / Elastosis perforans serpiginosa and pseudo-pseudoxanthomaelasticum secondary to D-penicillamine in a patient with Wilson’s disease

La D-penicilamina es la opción terapéutica más utilizada en la enfermedad de Wilson, rara enfermedad genética, autosómica recesiva, en la cual existe una alteración en el metabolismo del cobre que se deposita en los tejidos (hígado, encéfalo y córnea). Presenta numerosos efectos adversos, la mayoría cutáneos, que se observan cuando la droga es utilizada en altas dosis y por largo tiempo; entre ellos se encuentran las dermatosis degenerativas, que incluyen elastosis perforante serpiginosa, cutis laxa, anetodermia y pseudo-pseudoxantoma elástico (también llamado pseudoxantoma elástico símil o pseudoxantoma elástico like). Se presenta una paciente de 29 años con antecedentes de enfermedad de Wilson asociada a elastosis perforante serpiginosa y pseudo-pseudoxantoma elástico, ambas secundarias al tratamiento con D-penicilamina.

Penicillamineis the most commonly used therapeutic option in Wilson's disease.This is a rare, genetic, autosomal recessive diseasein which there is an alteration inthe metabolism of copper that is deposited in the tissues (liver, brain and cornea).It has numerous adverse effects, most of them affecting skin, but they are onlyobserved when the drug is used in high doses and for a long time, such as perforatingelastosis serpiginosa, cutis laxa, anetodermia and pseudo-pseudoxantomaelasticum (also called elasticum pseudoxantoma simil or elasticum pseudoxantomalike). We present the case of a29 year-old woman with a history of Wilson's diseaseand two concomitant degenerative dermatoses: elastosis perforans serpiginosa andpseudo pseudoxanthoma elasticum, both of them, secondary to treatment with Dpenicillamine.

Sífilis maligna en un paciente con infección por VIH: Presentación de un caso y revisión de la literatura / Malignant syphilis in a patient infected by human immunodeficiency virus: Case report and literature review

Malignant syphilis is a rare form of secondary syphilis strongly associated with human immunodeficiency virus infection (HIV). This clinical form of the disease is characterized by atypical cutaneous ulcerative and disseminated lesions and systemic compromise that can delay the final diagnosis. There are only few reports in the medical literature about malignant lues in HIV-infected patients. Malignant syphilis should be considered in the differential diagnosis in HIV-infected patients with fever and ulcerative skin lesions. Here we describe a man who developed clinical cutaneous and systemic manifestations pathologically conirmed as malignant syphilis and we performed a review of the literature.

La sífilis maligna es una forma rara de presentación de lúes secundaria asociada a la infección por el virus de la inmunodeficiencia humana (VIH). Se caracteriza por lesiones cutáneas atípicas, ulceradas, costrosas y diseminadas, asociadas con síntomas generales inespecíficos que pueden retrasar el diagnóstico correcto. Existen sólo escasas publicaciones en la literatura médica acerca de sífilis maligna en pacientes con infección por VIH. La lúes maligna debe incluirse en el diagnóstico diferencial de los pacientes con VIH que consultan por fiebre y lesiones úlcero-costrosas diseminadas. Se describe el caso de un paciente con infección por VIH que desarrolló una sífilis maligna con confirmación diagnóstica a partir de los hallazgos histopatológicos y se realiza una revisión de la literatura científica sobre el tema.