RESUMEN
Antiphospholipid syndrome (APS) is an acquired thrombophilic disorder in which autoantibodies are produced against a variety of phospholipids and phospholipid-binding proteins. The purpose of this article is to review cutaneous findings in patients with APS diagnosis. An overview regarding prevalence, description, pathogenesis and histopathology, are described for cutaneous manifestations of APS.
Asunto(s)
Anticuerpos Antifosfolípidos/inmunología , Síndrome Antifosfolípido/patología , Livedo Reticularis/patología , Enfermedades de la Piel/patología , Vasculitis/patología , Adulto , Anciano , Anetodermia/etiología , Anetodermia/patología , Anticuerpos Anticardiolipina/inmunología , Síndrome Antifosfolípido/diagnóstico , Síndrome Antifosfolípido/epidemiología , Síndrome Antifosfolípido/inmunología , Biopsia , Femenino , Gangrena/etiología , Gangrena/patología , Humanos , Livedo Reticularis/diagnóstico , Livedo Reticularis/etiología , Livedo Reticularis/inmunología , Inhibidor de Coagulación del Lupus/inmunología , Masculino , Papulosis Atrófica Maligna/etiología , Papulosis Atrófica Maligna/patología , Persona de Mediana Edad , Necrosis/diagnóstico , Necrosis/etiología , Prevalencia , Enfermedades de la Piel/inmunología , Úlcera/patología , Vasculitis/etiologíaRESUMEN
OBJECTIVES: To identify the aggregation of patients with aPL into different subgroups sharing common features in terms of clinical and laboratory phenotypes. METHODS: We applied a hierarchical cluster analysis from the multiple correspondence analysis to determine subgroups of patients according to clinical and laboratory characteristics in a cohort of subjects with confirmed aPL positivity who presented to our outpatient clinics from 2006 to 2018. RESULTS: A total of 486 patients [403 women; age 41.7 years (26)] were included, resulting in five clusters. Cluster 1 (n= 150) presented with thrombotic events (65.3% with venous thrombosis), with triple aPL positivity found in 34.7% of them (the highest rate among the different clusters). All the patients from cluster 2 (n = 91) had a confirmed diagnosis of SLE and the highest rate of anti-dsDNA positivity (91.7%). Cluster 3 included 79 women with pregnancy morbidity. Triple positivity was present in 3.8%, significantly lower when compared with Cluster 1 (34.7% versus 3.8%, P <0.01). Cluster 4 included 67 patients, 28 (41.8%) of whom with APS. Thrombotic events were observed in 23.9% patients. Cluster 4 had the highest rate of cytopenia, with thrombocytopenia as high 41.8% with no anti-dsDNA antibodies. Cluster 5 included 94 asymptomatic aPL carriers. CONCLUSION: While clusters 1, 2, 3 and 5 corresponded to well-known entities, cluster 4 might represent a bridging condition between pure primary APS and defined SLE, with lower thrombotic risk when compared with primary APS but higher general features such as ANA and cytopenia (mainly thrombocytopenia).
Asunto(s)
Anticuerpos Antifosfolípidos/sangre , Adolescente , Adulto , Anciano , Anticuerpos Anticardiolipina/sangre , Anticuerpos Antinucleares/sangre , Síndrome Antifosfolípido/inmunología , Análisis por Conglomerados , Estudios de Cohortes , Femenino , Humanos , Inmunoglobulina G/inmunología , Inmunoglobulina M/inmunología , Leucopenia/inmunología , Livedo Reticularis/inmunología , Lupus Eritematoso Sistémico/inmunología , Masculino , Persona de Mediana Edad , Fenotipo , Embarazo , Complicaciones del Embarazo/inmunología , Estudios Retrospectivos , Trombocitopenia/inmunología , Trombosis/inmunología , Adulto JovenRESUMEN
BACKGROUND: There are two distinctive acral manifestations of COVID-19 embodying disparate clinical phenotypes. One is perniosis occurring in mildly symptomatic patients, typically children and young adults; the second is the thrombotic retiform purpura of critically ill adults with COVID-19. OBJECTIVES: To compare the clinical and pathological profiles of these two different cutaneous manifestations of COVID-19. METHODS: We compared the light microscopic, phenotypic, cytokine and SARS-CoV-2 protein and RNA profiles of COVID-19-associated perniosis with that of thrombotic retiform purpura in critical patients with COVID-19. RESULTS: Biopsies of COVID-19-associated perniosis exhibited vasocentric and eccrinotropic T-cell- and monocyte-derived CD11c+ , CD14+ and CD123+ dendritic cell infiltrates. Both COVID-associated and idiopathic perniosis showed striking expression of the type I interferon-inducible myxovirus resistance protein A (MXA), an established marker for type I interferon signalling in tissue. SARS-CoV-2 RNA, interleukin-6 and caspase 3 were minimally expressed and confined to mononuclear inflammatory cells. The biopsies from livedo/retiform purpura showed pauci-inflammatory vascular thrombosis without any MXA decoration. Blood vessels exhibited extensive complement deposition with endothelial cell localization of SARS-CoV-2 protein, interleukin-6 and caspase 3; SARS-CoV-2 RNA was not seen. CONCLUSIONS: COVID-19-associated perniosis represents a virally triggered exaggerated immune reaction with significant type I interferon signaling. This is important to SARS-CoV-2 eradication and has implications in regards to a more generalized highly inflammatory response. We hypothesize that in the thrombotic retiform purpura of critically ill patients with COVID-19, the vascular thrombosis in the skin and other organ systems is associated with a minimal interferon response. This allows excessive viral replication with release of viral proteins that localize to extrapulmonary endothelium and trigger extensive complement activation.
Asunto(s)
COVID-19/complicaciones , Eritema Pernio/diagnóstico , Livedo Reticularis/diagnóstico , Púrpura/diagnóstico , SARS-CoV-2/inmunología , Adolescente , Factores de Edad , Anciano , Biopsia , COVID-19/diagnóstico , COVID-19/inmunología , COVID-19/virología , Caspasa 3/inmunología , Caspasa 3/metabolismo , Eritema Pernio/inmunología , Eritema Pernio/patología , Diagnóstico Diferencial , Femenino , Pie , Mano , Humanos , Interferón Tipo I/inmunología , Interferón Tipo I/metabolismo , Interleucina-6/inmunología , Interleucina-6/metabolismo , Livedo Reticularis/inmunología , Livedo Reticularis/patología , Livedo Reticularis/virología , Masculino , Persona de Mediana Edad , Proteínas de Resistencia a Mixovirus/análisis , Proteínas de Resistencia a Mixovirus/metabolismo , Púrpura/inmunología , Púrpura/patología , Púrpura/virología , ARN Viral/aislamiento & purificación , SARS-CoV-2/genética , SARS-CoV-2/aislamiento & purificación , Índice de Severidad de la Enfermedad , Piel/inmunología , Piel/patología , Piel/virología , Glicoproteína de la Espiga del Coronavirus/inmunología , Glicoproteína de la Espiga del Coronavirus/aislamiento & purificaciónRESUMEN
Neonatal Antiphospholipid syndrome (APS) is a rare disease related to transplacental passage of antiphospholipid (aPL) antibodies from the mother or de novo production of aPL in a newborn. Neonatal aPL antibodies have rarely been associated with thrombosis. We describe a 5-week-old infant who developed fever, portal vein thrombosis and livedo reticularis like skin rash. Evaluation for thrombosis revealed high titers of antiphospholipid (aPL) antibodies (dual positive) in the child without any evidence of aPL antibodies in the mother, suggesting a de novo production in the child. Autopsy findings revealed umbilical vein sepsis with thrombosis of portal vein secondary to gram positive cocci which led to multiple liver and lung abscesses. Additionally, the baby had disseminated Cytomegalovirus (CMV) disease (acquired postnatally) involving walls of umbilical and portal vein, liver, lungs, adrenals, pancreas, thymus, and kidneys. Our case highlights the need for testing of aPL in every neonate with arterial or venous thrombosis even when the mother may have no features suggestive of an autoimmune disease.
Asunto(s)
Anticuerpos Antifosfolípidos/sangre , Síndrome Antifosfolípido/inmunología , Livedo Reticularis/inmunología , Trombosis de la Vena/inmunología , Síndrome Antifosfolípido/patología , Autopsia , Resultado Fatal , Femenino , Humanos , Recién Nacido , Livedo Reticularis/patología , Vena Porta , Sepsis/patología , Trombosis de la Vena/patologíaAsunto(s)
Betacoronavirus/inmunología , Complemento C4b/inmunología , Infecciones por Coronavirus/complicaciones , Livedo Reticularis/diagnóstico , Fragmentos de Péptidos/inmunología , Neumonía Viral/complicaciones , Adolescente , Betacoronavirus/aislamiento & purificación , Biopsia , COVID-19 , Niño , Complemento C4b/análisis , Infecciones por Coronavirus/inmunología , Infecciones por Coronavirus/virología , Femenino , Humanos , Livedo Reticularis/inmunología , Masculino , Pandemias , Fragmentos de Péptidos/análisis , Neumonía Viral/inmunología , Neumonía Viral/virología , SARS-CoV-2 , Hermanos , Piel/inmunología , Piel/patología , Factores de TiempoRESUMEN
Sneddon's syndrome is a rare disease characterized by cerebrovascular events and livedo racemosa. There are often autoimmunological comorbidities, especially antiphospholipid antibody syndrome. The underlying pathophysiology is still not fully clarified. A causal therapy does not exist. The reported case shows a patient with a thrombophilic form of Sneddon's syndrome with the main symptoms of headache and thromboembolic events. Symptoms, laboratory parameters, histology and differential diagnoses are explained.
Asunto(s)
Trastornos Cerebrovasculares/complicaciones , Cefalea/complicaciones , Livedo Reticularis/complicaciones , Síndrome de Sneddon/complicaciones , Trastornos Cerebrovasculares/inmunología , Diagnóstico Diferencial , Humanos , Livedo Reticularis/inmunología , Síndrome de Sneddon/inmunología , Tromboembolia/complicacionesAsunto(s)
Livedo Reticularis/diagnóstico , Poliarteritis Nudosa/diagnóstico , Vasculitis/diagnóstico , Anciano , Anticuerpos Anticitoplasma de Neutrófilos/inmunología , Humanos , Livedo Reticularis/inmunología , Masculino , Peroxidasa/inmunología , Poliarteritis Nudosa/inmunología , Vasculitis/inmunologíaRESUMEN
Livedoid vasculopathy is a rare thrombotic cutaneous disease. This observational study aimed to assess the clinical and biological features of livedoid vasculopathy and the efficacy of treatments. Patients enrolled had typical livedoid vasculopathy both clinically and histologically. Investigation of thrombophilia was performed. Electromyography was undertaken in the presence of symptoms suggesting peripheral neuropathy. Eighteen women and 8 men were included, with a mean age of 35.5 years at onset. Twenty patients had at least one thrombophilia factor. Ten patients had a peripheral neuropathy with 2 of these patients demonstrating a specific thrombo-occlusive vasculopathy on muscle biopsy. Anticoagulation with low molecular weight heparin was the most prescribed therapy and was associated with the best outcome (effective in 14 patients). Eight patients had severe disease refractory to anticoagulation and required intravenous immunoglobulins, producing a good response in 6 patients.
Asunto(s)
Anticoagulantes/administración & dosificación , Heparina de Bajo-Peso-Molecular/administración & dosificación , Inmunoglobulinas Intravenosas/administración & dosificación , Factores Inmunológicos/administración & dosificación , Livedo Reticularis/tratamiento farmacológico , Administración Intravenosa , Administración Oral , Adolescente , Adulto , Anciano , Coagulación Sanguínea/efectos de los fármacos , Niño , Femenino , Francia/epidemiología , Humanos , Livedo Reticularis/sangre , Livedo Reticularis/epidemiología , Livedo Reticularis/inmunología , Masculino , Persona de Mediana Edad , Enfermedades del Sistema Nervioso Periférico/epidemiología , Factores de Riesgo , Trombofilia/sangre , Trombofilia/tratamiento farmacológico , Trombofilia/epidemiología , Factores de Tiempo , Resultado del Tratamiento , Adulto JovenAsunto(s)
Frío/efectos adversos , Células Endoteliales/metabolismo , Interleucina-8/sangre , Livedo Reticularis/sangre , Microvasos/metabolismo , Infiltración Neutrófila , Receptores de Interleucina-8B/metabolismo , Estaciones del Año , Úlcera Cutánea/sangre , Piel/irrigación sanguínea , Células Cultivadas , Quimiocina CXCL1/metabolismo , Células Endoteliales/inmunología , Células Endoteliales/patología , Humanos , Interleucina-8/genética , Livedo Reticularis/genética , Livedo Reticularis/inmunología , Livedo Reticularis/patología , Microvasos/inmunología , Microvasos/patología , Receptores de Interleucina-8B/inmunología , Piel/inmunología , Piel/patología , Úlcera Cutánea/inmunología , Úlcera Cutánea/patología , Regulación hacia ArribaRESUMEN
Livedo reticularis is a common cutaneous manifestation of APS and may be a prognostic marker of more severe disease. It is associated with arterial and venous thrombosis and pregnancy morbidity irrespective of the presence of antiphospholipid antibodies. Recent results suggest the possibility of an association with accelerated atherosclerosis in patients with livedo. Given the similarities between APS and livedo (aPL negative), experts in this field believe that livedo may represent the so-called seronegative antiphospholipid syndrome, although the exact relationship of livedo with seronegative APS remains to be elucidated. LV may present as painful cutaneous ulcers that are often difficult to treat. The underlying pathology involves prothrombotic as well as immunological processes with some overlap with APS. Treatment remains challenging and results are often variable.
Asunto(s)
Anticuerpos Anticardiolipina/sangre , Anticuerpos Antifosfolípidos/sangre , Livedo Reticularis , Complicaciones del Embarazo/inmunología , Adulto , Síndrome Antifosfolípido/complicaciones , Síndrome Antifosfolípido/inmunología , Aterosclerosis/diagnóstico , Aterosclerosis/fisiopatología , Diagnóstico Diferencial , Manejo de la Enfermedad , Femenino , Humanos , Livedo Reticularis/diagnóstico , Livedo Reticularis/etiología , Livedo Reticularis/inmunología , Embarazo , Pronóstico , Trombosis/complicacionesRESUMEN
Livedoid vasculopathy (LV) is a thrombotic vasculopathy of the skin of unknown origin. No treatment has been validated in this indication, but case reports demonstrated successful use of intravenous immunoglobulins (IVIg) in LV. We assessed the efficacy and tolerability of 2 g/kg IVIg therapy every month for 2â¼3 cycles in patients with refractory LV. We analyzed the efficacy, side effects and recurrence after long-term follow-up (51.9 ± 14.0 months) in seven patients with LV treated with 2 g/kg of IVIg. Mean clinical score of sum of erythema, ulceration and pain index (each: 0-3) was 5.7 ± 0.9 before the therapy and significantly lower after therapy (1.1 ± 0.5) (p = 0.001). Even after just one cycle of IVIg, the score decreased significantly from 5.7 ± 0.9 to 3.7 ± 0.9 (p = 0.002), especially the pain score. In one patient, LV has not recurred for over 7 years; six patients experienced recurrence after a mean of 12.7 ± 2.8 months. Out of the six patients, two patients were re-administered IVIg whereas the others were well controlled by conventional therapy. We propose that IVIg is a rapid, effective, and safe therapeutic option in LV refractory to other treatment modalities.
Asunto(s)
Inmunoglobulinas Intravenosas/administración & dosificación , Factores Inmunológicos/administración & dosificación , Livedo Reticularis/tratamiento farmacológico , Adolescente , Adulto , Femenino , Estudios de Seguimiento , Humanos , Inmunoglobulinas Intravenosas/efectos adversos , Factores Inmunológicos/efectos adversos , Livedo Reticularis/inmunología , Livedo Reticularis/patología , Masculino , Quimioterapia por Pulso , Recurrencia , Retratamiento , Resultado del Tratamiento , Adulto JovenRESUMEN
Livedo vasculopathy (LV) is a chronic cutaneous disorder characterised by recurrent, painful ulceration ending in stellate scars. We have conducted a retrospective study of clinical features and treatment response of LV in 24 Chinese patients. LV occurred more frequently in women (male:female ratio 1:3). The peak age at onset of disease ranged from 14 to 20 years, younger than previously published data. 87.5% of the patients (21/24) showed significant summer exacerbation with ulcer formation. Out of 24 patients tested, 14 (58.3%) had positive antiphospholipid antibodies. Ten out of 14 patients (71.4%) were tested to be hypersensitive to multivalent insect antigens. Combinative anti-inflammatory therapy with steroids, tetracycline and Tripterygium glycosides plus antiplatelet/profibrinolytic drugs promoted quick healing of ulcer and reduce recurrence. The younger age of disease presentation and significant summer exacerbation are 2 novel clinical features observed in this study. These findings suggest that apart from procoagulation other risk factors may contribute significantly to the pathogenesis of LV. Although antiplatelet/profibrinolytic drugs are deemed as a first line therapy for LV, anti-inflammatory medications such as steroids, tetracycline and Tripterygium glycosides, from our experiences, are indispensable, especially for acute, ulcerative stage of disease.
Asunto(s)
Úlcera de la Pierna/tratamiento farmacológico , Úlcera de la Pierna/patología , Livedo Reticularis/tratamiento farmacológico , Livedo Reticularis/patología , Adolescente , Adulto , Distribución por Edad , Animales , Antibacterianos/uso terapéutico , Antiinflamatorios/uso terapéutico , Anticuerpos Antifosfolípidos/sangre , Pueblo Asiatico , Biopsia , China , Femenino , Fibrinolíticos/uso terapéutico , Humanos , Hipersensibilidad/inmunología , Mordeduras y Picaduras de Insectos/inmunología , Úlcera de la Pierna/inmunología , Livedo Reticularis/inmunología , Masculino , Fitoterapia , Inhibidores de Agregación Plaquetaria/uso terapéutico , Prednisona/uso terapéutico , Estudios Retrospectivos , Estaciones del Año , Distribución por Sexo , Piel/patología , Tetraciclina/uso terapéutico , Tripterygium , Adulto JovenRESUMEN
BACKGROUND: Livedoid vasculopathy (LV) is a thrombotic vasculopathy of the skin of unknown origin. No treatment has been validated in this indication, but case reports suggest the successful use of intravenous immunoglobulins (IVIG) in LV. METHODS: Outcomes in five patients treated with IVIG for treatment-resistant ulcerated LV were retrospectively analyzed. RESULTS: Treatment with IVIG induced complete remission (based on clinical evaluation and a pain-related visual analog scale) in four patients but was ineffective in one patient. Three patients relapsed; the median time to relapse was 10.7 months. Re-treatment with IVIG in these three patients was successful. CONCLUSIONS: These cases confirm previous reports that IVIG seems to be a rapid, effective, and safe treatment for patients with idiopathic refractory ulcerated LV. However, a placebo-controlled study is mandatory to confirm these results.
Asunto(s)
Inmunoglobulinas Intravenosas/administración & dosificación , Livedo Reticularis/terapia , Úlcera Cutánea/terapia , Trombosis/terapia , Adulto , Anciano , Femenino , Humanos , Livedo Reticularis/inmunología , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Úlcera Cutánea/inmunología , Trombosis/inmunología , Adulto JovenRESUMEN
OBJECTIVES: The aim of this study was to investigate the importance of aPL type and level for non-criteria-related events in APS patients. METHODS: Our study included 374 patients: 260 with PAPS and 114 with APS associated with systemic lupus erythematosus (SLE). RESULTS: We discovered significant connection between migraine and LA absence, livedo reticularis and aCL-IgG, skin ulcerations with aCL-IgG and anti-ß2GPI-IgM, pseudovasculitis lesions with aCL-IgG, aCL-IgM and anti-ß2GPI-IgM, and thrombocytopenia with aCL-IgM, aCL-IgG and anti-ß2GPI-IgG. Thrombocytopenia occurred more frequently in patients with more than one aPL. In PAPS, epilepsy correlated with ß2GPI-IgM, migraine with aCL-IgM, and thrombocytopenia with aCL-IgM, aCL-IgG, anti ß2GPI-IgG and LA. Skin ulcerations occurred more frequently in IIc category patients and in patients with high levels of aCL-IgG and anti ß2GPI-IgG. Livedo reticularis was more prominent in PAPS with high levels of aCL-IgG. Significantly higher prevalence of thrombocytopenia was observed in patients with high levels of aCL-IgG and anti ß2GPI-IgG. Epilepsy was related to high levels of anti ß2GPI-IgM and thrombocytopenia in the SAPS was correlated with aCL-IgG. Skin ulcerations were more prevalent in aCL-IgM positive SAPS patients and epilepsy more frequently in SAPS patients with high levels of anti ß2GPI-IgG. CONCLUSIONS: Our study showed that certain aPL type with certain level correlated with non-criteria manifestations, suggesting their predictive role.
Asunto(s)
Anticuerpos Antifosfolípidos/sangre , Síndrome Antifosfolípido/epidemiología , Síndrome Antifosfolípido/inmunología , Lupus Eritematoso Sistémico/epidemiología , Lupus Eritematoso Sistémico/inmunología , Adulto , Anticuerpos Anticardiolipina/sangre , Síndrome Antifosfolípido/sangre , Síndrome Antifosfolípido/diagnóstico , Biomarcadores/sangre , Distribución de Chi-Cuadrado , Femenino , Humanos , Inmunoglobulina G/sangre , Inmunoglobulina M/sangre , Livedo Reticularis/epidemiología , Livedo Reticularis/inmunología , Inhibidor de Coagulación del Lupus/sangre , Lupus Eritematoso Sistémico/sangre , Lupus Eritematoso Sistémico/diagnóstico , Masculino , Persona de Mediana Edad , Trastornos Migrañosos/epidemiología , Trastornos Migrañosos/inmunología , Valor Predictivo de las Pruebas , Prevalencia , Estudios Prospectivos , Serbia/epidemiología , Úlcera Cutánea/epidemiología , Úlcera Cutánea/inmunología , Trombocitopenia/epidemiología , Trombocitopenia/inmunología , Vasculitis/epidemiología , Vasculitis/inmunología , beta 2 Glicoproteína I/inmunologíaAsunto(s)
Anticuerpos Antifosfolípidos , Síndrome Antifosfolípido/patología , Lupus Eritematoso Sistémico/patología , Adulto , Síndrome Antifosfolípido/inmunología , Estudios Transversales , Femenino , Humanos , Livedo Reticularis/inmunología , Livedo Reticularis/patología , Lupus Eritematoso Sistémico/inmunología , Masculino , Enfermedades Cutáneas Vasculares/inmunología , Enfermedades Cutáneas Vasculares/patología , Úlcera Cutánea/inmunología , Úlcera Cutánea/patologíaRESUMEN
Livedo vasculopathy is characterized by reticular distribution of purpuric macules and papules of the lower legs, caused by intraluminal thrombosis of small vessels. Antiphospholipid antibodies are detected in a subset of these patients. We treated two cases (a 34-year-old man and a 46-year-old woman) with livedo vasculopathy. In both cases, thrombosis was seen only in the skin. The presence of immunoglobulin (Ig)G or IgM anticardiolipin antibody (Ab), IgG or IgM anti-ß(2) -glycoprotein I Ab, or lupus anticoagulant are necessary for criteria-based diagnosis of antiphospholipid syndrome. However, our patients were negative for these Ab, and instead had either IgG antiphosphatidylethanolamine Ab or IgA anticardiolipin Ab. These Ab are suggestive of antiphospholipid syndrome but are not considered "criteria" Ab. This report demonstrates the existence of antiphosphatidylethanolamine Ab or IgA anticardiolipin Ab in patients with livedo vasculopathy. However, the frequency and significance of these Ab in livedo vasculopathy should be confirmed in larger longitudinal studies.
Asunto(s)
Anticuerpos Anticardiolipina/sangre , Inmunoglobulina A/sangre , Inmunoglobulina G/sangre , Livedo Reticularis/inmunología , Fosfatidiletanolaminas/inmunología , Adulto , Femenino , Humanos , Livedo Reticularis/patología , Masculino , Persona de Mediana EdadRESUMEN
Antiphospholipid syndrome is characterized by arterial and venous thromboembolic events and persistent laboratory evidence of antiphospholipid antibodies. Obstetric complications such as recurrent miscarriage, early delivery, oligohydramnios, prematurity, intrauterine growth restriction, fetal distress, fetal or neonatal thrombosis, pre-eclampsia/eclampsia, and HELLP syndrome are also hallmarks of antiphospholipid syndrome. This syndrome is one of the diseases associated with the most severe thrombotic risk. Changes in the hemostatic system during normal pregnancy also result in a hypercoagulable state resulting in elevated thrombotic risk. Thromboembolic events are responsible of the vast majority of maternal and fetal deaths. Administration of appropriate thromboprophylaxis helps prevent thromboembolic complications during pregnancy in women with antiphospholipid syndrome and also give birth to healthy children. It is important to centralize the medication and management of these patients. It helps in the thoughtful care of these pregnant women encountering serious problems.
Asunto(s)
Anticuerpos Antifosfolípidos/sangre , Anticoagulantes/uso terapéutico , Síndrome Antifosfolípido/diagnóstico , Síndrome Antifosfolípido/inmunología , Complicaciones del Embarazo/inmunología , Tromboembolia/prevención & control , Aborto Habitual/inmunología , Aborto Habitual/prevención & control , Anticoagulantes/efectos adversos , Síndrome Antifosfolípido/complicaciones , Susceptibilidad a Enfermedades , Femenino , Retardo del Crecimiento Fetal/inmunología , Síndrome HELLP/inmunología , Heparina/uso terapéutico , Humanos , Livedo Reticularis/inmunología , Osteoporosis/inducido químicamente , Preeclampsia/inmunología , Embarazo , Atención Prenatal , Índice de Severidad de la Enfermedad , Mortinato , Trombocitopenia/inducido químicamente , Tromboembolia/inmunologíaRESUMEN
A wide variety of dermatologic manifestations has been described in the antiphospholipid syndrome (APS). The most frequent skin lesion is livedo reticularis, present not only on the limbs but also on the trunk, with a fine irregular pattern. It belongs to the arterial subset of APS. Circumscribed ulcerations, resembling livedoid vasculitis, may be the first manifestation of APS. Ulcerations may also occur as a late complication of recurrent venous thrombosis. Extensive skin necrosis is a classic manifestation of catastrophic APS. Pseudo-vasculitis lesions are misdiagnosed if a skin biopsy is not performed, especially in the context of systemic lupus erythematosus. In systemic lupus erythematosus, primary anetoderma is always associated with antiphospholipid antibodies.
Asunto(s)
Síndrome Antifosfolípido/complicaciones , Enfermedades de la Piel/inmunología , Anetodermia/inmunología , Anticuerpos Monoclonales de Origen Murino/uso terapéutico , Anticoagulantes/uso terapéutico , Síndrome Antifosfolípido/diagnóstico , Síndrome Antifosfolípido/inmunología , Síndrome Antifosfolípido/terapia , Quimioterapia Combinada , Glucocorticoides/uso terapéutico , Heparina/uso terapéutico , Humanos , Factores Inmunológicos/uso terapéutico , Inmunosupresores/uso terapéutico , Livedo Reticularis/inmunología , Intercambio Plasmático/métodos , Rituximab , Enfermedades de la Piel/diagnóstico , Enfermedades de la Piel/terapia , Enfermedades Cutáneas Vasculares/inmunología , Resultado del Tratamiento , Vasculitis/etiologíaRESUMEN
One of identical twin girls was born with ulcers on her leg, and shortly after birth developed a flaccid blister on the leg. Subepidermal blister with vacuolar degeneration of basal cell layer and the heavy infiltration of mononuclear cells in the upper dermis were observed in the blister lesion. She also had generalized livedo. Her identical twin sister did not exhibit ulcers or blisters, but was born with milia on her limbs. Their mother was found to have lupus erythematosus with positive anti-Ro/SSA antibodies and developed Sjögren syndrome. We emphasize neonatal blistering and congenital milia unique manifestations of neonatal lupus erythematosus.