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Tromboflebitis , Humanos , Tromboflebitis/etiología , Tromboflebitis/patología , Tromboflebitis/diagnóstico , Eritema/patología , Eritema/etiología , Arteritis/patología , Síndrome de Churg-Strauss/complicaciones , Síndrome de Churg-Strauss/diagnóstico , Síndrome de Churg-Strauss/patología , Femenino , Masculino , Granulomatosis con Poliangitis/patología , Granulomatosis con Poliangitis/complicaciones , Granulomatosis con Poliangitis/diagnóstico , Livedo Reticularis/patología , Livedo Reticularis/etiología , Persona de Mediana EdadRESUMEN
INTRODUCTION: Cutis marmorata telangiectatica congenita (CMTC) is a rare capillary malformation characterized by persistent reticular and violaceous erythema. We present two cases of CMTC. CLINICAL OBSERVATION: The first case involved a 13-month-old male with a reticular violaceous macule on the left gluteal region and a brownish papule with Darier's sign on the inner malleolus of the left foot, which was biopsied, revealing > 15 mast cells per field, leading to a diagnosis of CMTC and solitary cutaneous mastocytoma. The second case involved a newborn with a characteristic CMTC lesion without other malformations at birth, who subsequently developed two cutaneous tumors consistent with infantile hemangiomas during follow-up. DISCUSSION: CMTC is a benign condition. However, approximately 50% of cases exhibit associated anomalies. When CMTC is suspected, musculoskeletal, ophthalmological, and cutaneous malformations should be ruled out. To the best of our knowledge, this is the first report of CMTC associated with mastocytoma and one of the few cases associated with infantile hemangioma.
INTRODUCCION: La cutis marmorata telangiectásica congénita (CMTC) es una rara malformación capilar caracterizada por eritema reticular y violáceo persistente. Presentamos dos casos de CMTC. OBSERVACION CLINICA: Un varón de 13 meses presentaba una mácula violácea reticular en glúteo izquierdo y una pápula parduzca con signo de Darier en el maléolo interno del pie izquierdo, que fue biopsiada identificando > 15 mastocitos/campo, con lo cual se diagnosticó de CMTC y mastocitoma cutáneo solitario. El segundo caso, una recién nacida con una lesión característica de CMTC sin otras malformaciones al nacer, que durante el seguimiento desarrolló dos tumoraciones cutáneas compatibles con hemangiomas infantiles. COMENTARIOS: La CMTC es una condición benigna, sin embargo, aproximadamente el 50% de los casos presentan anomalías asociadas. Ante la sospecha de CMTC se deben descartar malformaciones musculoesqueléticas, oftalmológicas y cutáneas. Hasta donde tenemos conocimiento, este es el primer reporte de CMTC asociada con mastocitoma y uno de los pocos con hemangioma infantil.
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Livedo Reticularis , Mastocitoma , Recién Nacido , Masculino , Humanos , Lactante , Biopsia , Livedo Reticularis/etiologíaRESUMEN
Sickle cell disease is a monogenic hemoglobinopathy that results in the abnormal production of hemoglobin S, which yields the characteristic sickle-shaped red blood cells. Sickle cell vaso-occlusive crisis is a painful complication of sickle cell disease caused by red blood cell entrapment within the microcirculation. The resulting tissue ischemia triggers a secondary inflammatory process involved in the pathogenesis of varying inflammatory skin conditions. Chronic leg ulcers are the most common skin presentation in sickle cell disease. A 58-year-old woman with sickle cell disease presented with systemic edematous plaques with the most notable involvement of her bilateral legs, which exhibited reticulated purpuric patches with central pallor. We report a case highlighting an unusual presentation of livedo racemosa as the presenting sign in a patient with sickle cell disease in vaso-occlusive crisis.
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Anemia de Células Falciformes , Livedo Reticularis , Humanos , Femenino , Persona de Mediana Edad , Livedo Reticularis/etiología , Anemia de Células Falciformes/complicaciones , Dolor/etiologíaRESUMEN
Primary systemic vasculitis can present with a wide spectrum of manifestations ranging from systemic non-specific features such as fever, malaise, arthralgia and myalgia to specific organ damage. We describe two cases of cholesterol embolisation syndrome and Kaposi sarcoma mimicking primary systemic vasculitis, both of which were characterised by features such as livedo reticularis, blue toe syndrome, a brown purpuric skin rash and positive perinuclear anti-neutrophil cytoplasmic antibodies associated with Kaposi sarcoma. Establishing the right diagnosis was challenging and thus this report aimed to highlight the possible ways to distinguish them from primary systemic vasculitis.
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Síndrome del Dedo Azul , Livedo Reticularis , Sarcoma de Kaposi , Vasculitis Sistémica , Humanos , Síndrome del Dedo Azul/complicaciones , Sarcoma de Kaposi/diagnóstico , Sarcoma de Kaposi/complicaciones , Livedo Reticularis/etiología , Livedo Reticularis/patología , Vasculitis Sistémica/complicacionesRESUMEN
Lymphocytic thrombophilic arteritis and livedoid vasculopathy may both present with livedo racemosa and ulceration. We present 6 cases with features of both conditions, raising the possibility that they are either closely linked or are part of a spectrum of the same condition.
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Arteritis , Livedo Reticularis , Trombofilia , Humanos , Livedo Reticularis/etiología , Arteritis/complicaciones , Trombofilia/complicaciones , LinfocitosRESUMEN
We herein report a case of hepatitis C virus (HCV)-associated cryoglobulinemic livedo reticularis in a woman in her 60s that improved with direct-acting antivirals (DAAs). Hyperpigmentation was observed in both lower legs, and a skin biopsy confirmed livedo reticularis, suggesting a relationship with cryoglobulinemia and HCV infection. DAAs with an NS5A inhibitor+NS3/4A protease inhibitor (glecaprevir/pibrentasvir) were administered for eight weeks, and a sustained virological response (SVR) was obtained. The disappearance of serum cryoglobulin was confirmed approximately two years after an SVR was obtained and livedo reticularis was improved. DAA therapy can be an effective therapeutic option for extrahepatic complications associated with HCV infection.
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Crioglobulinemia , Hepatitis C Crónica , Hepatitis C , Livedo Reticularis , Femenino , Humanos , Antivirales , Hepacivirus , Hepatitis C Crónica/complicaciones , Hepatitis C Crónica/tratamiento farmacológico , Crioglobulinemia/complicaciones , Crioglobulinemia/tratamiento farmacológico , Livedo Reticularis/etiología , Livedo Reticularis/complicaciones , Hepatitis C/complicaciones , Hepatitis C/tratamiento farmacológicoRESUMEN
Livedo racemosa (LRac) refers to violaceous, red, or blue netlike mottling of the skin in an irregular pattern caused by circulatory abnormalities. Central venous catheters (CVC) are widely used in neonates who require intensive care to offer steady intravenous access. CVCs are covered with a hydrophilic polymer gel that reduces friction between the sheath and the vessel wall, thereby minimizing vascular spasm, irritation, and aneurysm formation. However, the introduction of foreign material into the vein carries a risk of embolization. Recently, LRac was reported as a cutaneous manifestation of hydrophilic polymer gel embolization (HPE) in adults. We present a case of LRac in a newborn that developed following CVC insertion and resolved spontaneously after the catheter was removed.
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Catéteres Venosos Centrales , Livedo Reticularis , Recién Nacido , Adulto , Humanos , Catéteres Venosos Centrales/efectos adversos , Livedo Reticularis/etiología , PolímerosRESUMEN
BACKGROUND: Antiphospholipid syndrome (APS) is characterized by antiphospholipid antibodies (aPLs) associated with thrombosis (arterial and/or venous) and/or obstetrical manifestations. However, various manifestations, which are considered to be noncriteria manifestations, are frequently found in APS. AIM: The purpose of this study was to evaluate whether noncriteria manifestations may be found more frequently in subjects with thrombotic and/or obstetrical APS ("criteria" manifestations) in a population of patients with primary APS (PAPS). This study presents the results from our national cohort. PATIENTS AND METHODS: This is a cross-sectional study of 360 PAPS patients. Data regarding the presence of thrombocytopenia, livedo reticularis, chorea, and valvulopathy were analyzed. The aPL analysis included the detection of anticardiolipin antibodies (aCLs: immunoglobulin G [IgG]/IgM), anti-ß 2 glycoprotein I (IgG/IgM), and lupus anticoagulant positivity. RESULTS: In our cohort, livedo reticularis was significantly related to arterial thromboses in the same way as valvular manifestations (valvular vegetations and valvular thickening and dysfunction not related to age) ( p = 0.0001, p = 0.013, respectively). Age was strongly related to all the noncriteria manifestations analyzed. Thrombocytopenia was significantly related to ß 2 glycoprotein I IgG and lupus anticoagulant positivity ( p = 0.043, p = 0.030, respectively), as well as to double and triple aPL positivity ( p = 0.041, p = 0.013 respectively). Moreover, in a multivariate model, livedo reticularis was strongly and independently related to arterial thrombosis in our cohort (odds ratio, 2.010; confidence interval, 1.229-3.288; p = 0.005). CONCLUSION: This cross-sectional analysis of a large cohort of Serbian PAPS patients confirmed a strong relationship between livedo reticularis and arterial thrombosis, suggesting a more cautious approach regarding the presence of noncriteria manifestations, especially livedo reticularis, in APS.
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Síndrome Antifosfolípido , Livedo Reticularis , Trombocitopenia , Trombosis , Anticuerpos Anticardiolipina/análisis , Anticuerpos Antifosfolípidos , Síndrome Antifosfolípido/complicaciones , Síndrome Antifosfolípido/diagnóstico , Síndrome Antifosfolípido/epidemiología , Estudios de Cohortes , Estudios Transversales , Humanos , Inmunoglobulina G , Inmunoglobulina M , Livedo Reticularis/diagnóstico , Livedo Reticularis/epidemiología , Livedo Reticularis/etiología , Inhibidor de Coagulación del Lupus , Serbia/epidemiología , Trombosis/diagnóstico , Trombosis/epidemiología , Trombosis/etiología , beta 2 Glicoproteína IRESUMEN
PURPOSE: To report on a patient with probable catastrophicantiphospholipid syndrome (CAPS) with eye, brain and skin involvement. METHODS: Descriptive presentation of the case and of the relevant clinical photographs. RESULTS: A 17-year-old girl presented with fatigue, loss of appetite, arthralgia, lower limb skin ulcers and livedo reticularis. Workup showed anemia, elevated ESR, CRP, and positive anti-phospholipid antibodies. Right eye funduscopy showed cotton-wool spots along the inferotemporal arcade with arteriolar occlusion, perivascular retinal hemorrhages, and diffuse retinal ischemia in the temporal peripheral retina. Fluorescein angiogram confirmed the widespread retinal ischemia. Brain MRI revealed several white matter lacunar infarcts, minute cortical/subcortical hemorrhages and subarachnoidal insular hemorrhage. The patient was treated with immunomodulatory therapy, Enoxaparin and retinal laser photocoagulation. Within 2 months of treatment, the facial livedo reticularis resolved and the leg ulcers markedly improved. CONCLUSION: CAPS causes multiple organ thrombosis and is associated with high rate of mortality.
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Síndrome Antifosfolípido , Livedo Reticularis , Enfermedades de la Retina , Adolescente , Anticuerpos Antifosfolípidos , Síndrome Antifosfolípido/complicaciones , Síndrome Antifosfolípido/diagnóstico , Encéfalo/diagnóstico por imagen , Ojo , Femenino , Humanos , Isquemia , Livedo Reticularis/complicaciones , Livedo Reticularis/etiología , PielRESUMEN
The term Sneddon's syndrome (SS) has been used since 1965 to describe a vasculopathy characterized by a combination of cerebrovascular disease with livedo racemosa. SS may be classified as antiphospholipid+ (aPL+) or antiphospholipid- (aPL-). Little is known about aPL- SS; in this review we describe the epidemiology and pathogenesis of aPL- SS, as well as the clinical and histologic features. We discuss recent findings in terms of neurologic and cardiac involvement. Moreover, differential diagnoses of conditions that may present with both livedo racemosa and stroke are discussed. Finally, we discuss real-life practical issues such as the initial investigations to be performed, long-term follow-up, and therapeutic management of aPL- SS patients.