Livedo reticularis y dengue

Se presenta a un varón de 40 años, residente en la ciudad de Lima, sin viajes recientes, con fiebre, malestar general, cefalea y diarrea. Acudió al Servicio de Emergencia y los exámenes auxilares mostraron leucopenia y trombocitopenia leve. Los estudios para Epstein-Barr, hepatitis B, toxoplasma, rubéola, citomegalovirus, herpes 1 y 2 y COVID-19 fueron negativos. Los anticuerpos IgM y IgG para dengue fueron negativos, y la proteína NS1 fue positiva. El paciente fue diagnosticado con dengue y solo recibió paracetamol. En el seguimiento, en el séptimo día de enfermedad, se le halló afebril y con lesiones dérmicas tipo livedo reticularis en los miembros, principalmente. Se resalta este inusual patrón cutáneo en dengue.

We present the case of a 40-year-old male, resident of Lima city, with no recent travels, with fever, general malaise, headache and diarrhea. He went to the Emergency Department and auxiliary tests showed leukopenia and mild thrombocytopenia. Studies for Epstein-Barr, hepatitis B, toxoplasma, rubella, CMV, herpes 1-2 and COVID-19 were negative. IgM and IgG antibodies for dengue were negative and NS1 protein was positive. He was diagnosed with dengue. He only received paracetamol. On follow-up, on the seventh day of illness, he was found afebrile and with livedo reticularis type dermal lesions on the limbs, mainly. This unusual cutaneous pattern in dengue is highlighted.

Cutaneous manifestations of COVID-19 / 中华皮肤科杂志

COVID-19 can be accompanied by a variety of cutaneous abnormalities, which mainly include vascular lesions (chilblain-like lesions, livedo reticularis, purpura, ecchymosis, acral cyanosis, gangrene, etc) and inflammatory lesions (diffuse erythema, morbilliform exanthem, acute urticaria, varicella-like exanthem, etc) . Some types of skin lesions may be the first symptom or the only clinical manifestation of COVID-19.

Analysis of clinical characteristics of 64 cases of livedoid vasculopathy / 中华皮肤科杂志

Objective:To analyze clinical features and distribution patterns of livedoid vasculopathy lesions, especially obvious livedo reticularis and purpuric lesions.Methods:Clinical data were collected from 64 patients with confirmed livedoid vasculopathy in Hospital of Dermatology, Chinese Academy of Medical Sciences from July 2017 to October 2020, and analyzed retrospectively.Results:Among the 64 patients, 23 were males and 41 were females, aged 13 - 54 years; their age at onset ranged from 7 to 51 years, and 48 developed livedoid vasculopathy before the age of 25 years; the course of disease ranged from 6 months to 10 years. Livedoid vasculopathy occurred or worsened in summer in 49 patients, and skin lesions mainly manifested as necrotic irregular purpura, purpuric dermatosis-like erythema, irregular ulcers, livedo reticularis, telangiectasia, irregular white atrophic scars and pigmentation. Among the 64 patients, ulcers and necrotic purpura were mostly irregular, and occurred on the dorsum of the foot and around the ankle. A total of 40 patients presented with purpuric dermatosis-like lesions, including 32 with pigmented purpura and 4 with telangiectatic purpura. Besides, numbness, tingling and other symptoms of nerve terminal damage occurred in 4 patients.Conclusion:Clinical manifestations of livedoid vasculopathy are diverse, and differential diagnosis is important for patients with generalized livedo reticularis, purpuric dermatosis-like lesions and symptoms such as numbness.

Enfermedad por aglutininas frías: proceso diagnóstico, tratamiento y seguimiento de un paciente / Cold agglutinin disease: diagnosis, treatment and follow-up of a patient

La enfermedad por crioaglutininas es una anemia hemolítica autoinmune que se caracteriza, en la gran mayoría de los casos, por la hemólisis mediada por autoanticuerpos de tipo IgM y complemento C3d, contra los antígenos de la membrana del eritrocito, que conduce a hemólisis extravascular con propensión a la trombosis, y que afecta principalmente al sexo femenino y personas mayores. Su diagnóstico se realiza con la prueba de Coombs directo y fraccionado, y la titulación de aglutininas frías >1:64 a 4 °C. Se describe el caso clínico de una mujer de 89 años con un síndrome constitucional y una anemia de 3 años de evolución, en quien se determinó el diagnóstico de enfermedad por aglutininas frías. Asimismo, se describe el abordaje diagnóstico, el tratamiento instaurado, y se hace una breve revisión de la literatura publicada

Cold agglutinin disease (CAD) is an autoimmune hemolytic anemia characterized in the vast majority of cases by hemolysis mediated by IgM autoantibodies and complement C3d against erythrocyte membrane antigens, leading to extravascular hemolysis with propensity to thrombosis, affecting mainly females and older individuals. It is diagnosed by direct and fractionated Coombs test and a cold agglutinin titer >1:64 at 4 °C. We describe the case of an 89-year-old woman with a constitutional syndrome and a 3-year history of anemia, who was diagnosed with cold agglutinin disease. Also, we include the diagnostic and treatment approach, and a brief review of the literature

Pancreatitis aguda grave en un paciente fumador / Serious acute pancreatitis in a smoking patient

Se describe el caso clínico de un paciente de 51 años de edad con antecedente patológico personal de ser un fumador empedernido, el cual acudió al Cuerpo de Guardia del Hospital Provincial Docente Dr. Joaquín Castillo Duany de Santiago de Cuba por presentar disnea asociada a cifras elevadas de tensión arterial y livedo reticular en la cara anterior abdominal. Debido al cuadro clínico y a los resultados de los exámenes complementarios, fue trasladado a la Unidad de Cuidados Intensivos, donde posteriormente se le diagnosticó pancreatitis aguda grave, tras realizar una laparotomía exploratoria y hallar zonas isquémicas en las asas intestinales. El paciente mantuvo una evolución tórpida y falleció a causa de una disfunción múltiple de órganos.

The case report of a 51 years patient with personal pathological history of being a heavy smoker is described, who went to the emergency room of Dr. Joaquín Castillo Duany Teaching Provincial Hospital in Santiago de Cuba due to a dyspnea associated with high figures of blood pressure and livedo reticularis in the abdominal anterior face. Due to the clinical pattern and the results of the complementary tests, he was referred to the Intensive Cares Unit, where later on he was diagnosed serious acute pancreatitis, after an exploratory laparotomy where ischemic areas in the intestinal loops were found. The patient maintained a torpid clinical course and he died because of a multiple dysfunction of organs.

Is macular lymphocytic arteritis limited to the skin? Long-term follow-up of seven patients

Abstract Background: Macular lymphocytic arteritis most commonly presents as hyperpigmented macules on the lower limbs. The pathogenesis of this disease is still unclear and there is an ongoing debate regarding whether it represents a new form of cutaneous vasculitis or an indolent form of cutaneous polyarteritis nodosa. Objective: To describe clinical, histopathological, and laboratory findings of patients with the diagnosis of macular lymphocytic arteritis. Methods: A retrospective search was conducted by reviewing cases followed at the Vasculitis Clinic of the Dermatology Department, School of Medicine, University of São Paulo, between 2005 and 2017. Seven patients were included. Results: All cases were female, aged 9-46 years, and had hyperpigmented macules mainly on the legs. Three patients reported symptoms. Skin biopsies evidencing a predominantly lymphocytic infiltrate affecting arterioles at the dermal subcutaneous junction were found, as well as a typical luminal fibrin ring. None of the patients developed necrotic ulcers, neurological damage, or systemic manifestations. The follow-up ranged from 18 to 151 months, with a mean duration of 79 months. Study limitations: This study is subject to a number of limitations: small sample of patients, besides having a retrospective and uncontrolled study design. Conclusions: To the best of the authors' knowledge, this series presents the longest duration of follow-up reported to date. During this period, none of the patients showed resolution of the lesions despite treatment, nor did any progress to systemic vasculitis. Similarities between clinical and skin biopsy findings support the hypothesis that macular lymphocytic arteritis is a benign, incomplete, and less aggressive form of cutaneous polyarteritis nodosa.

Vasculopatia livedoide tratada com rivaroxabana / Livedoid vasculopathy treated with rivaroxaban

A vasculopatia livedoide é uma doença rara caracterizada pela oclusão da microvasculatura da derme, originando lesões maculosas que, posteriormente, podem evoluir para úlceras e cicatrizes atróficas. Como um fenômeno vaso-oclusivo, o tratamento geralmente é realizado com antiplaquetários e fibrinolíticos. O presente relato descreve o caso de uma paciente refratária à terapia convencional, que obteve regressão da doença utilizando a rivaroxabana, um fármaco inibidor seletivo do fator Xa. (AU)

Livedoid vasculopathy is a rare disease characterized by occlusion of the dermis microvasculature, leading to spotted lesions that can later develop into ulcers and atrophic scars. As a vaso- occlusive phenomenon, treatment is usually performed with antiplatelet and fibrinolytic agents. The present report describes the case of a female patient refractory to conventional therapy who presented disease remission using rivaroxaban, a selective factor Xa inhibitor drug. (AU)

Livedo reticularis by hypothermia during anesthesia for dental treatment in Down's syndrome patient / Livedo reticular por hipotermia durante anestesia para tratamento odontológico em paciente com síndrome de Down

Abstract Background: Livedo reticularis is a benign dermatological condition characterized by ischemic areas permeated by erythematous-cyanotic areas in a lacy pattern, and may be transient or permanent and is frequently associated with body exposure to cold. Cutaneous arterial vasospasm promotes ischemia, and venous dilation of the congested areas occurs by tissue hypoxia or autonomic dysfunction. Patients with Down's syndrome, due to their physiological peculiarities, constitute a representative part of those who require dental care under general anesthesia, and livedo reticularis has a reported incidence of 8-12% in Down's syndrome patients. Objectives: To describe the physiological livedo reticularis in a Down's syndrome patient, with the onset during the anesthetic-surgical procedure. Case report: 5-year-old female patient with Down's syndrome, admitted for dental treatment under balanced general anesthesia with sevoflurane, fentanyl, and atracurium. Transoperative hypothermia occurred with axillary temperature reaching 34.5 °C after 30 min after the beginning of anesthesia. At the end of the procedure, red-purplish skin lesions interspersed with areas of pallor were observed exclusively on the ventromedial aspect of the right forearm, with no systemic signs suggestive of allergic reactions. The established diagnosis was physiological livedo reticularis. There was a total fading of the lesions within 5 days. Conclusion: This report evidences the need for thermal control of patients undergoing anesthesia, as well as the manifestation of livedo reticularis as a consequence of transoperative hypothermia.

Resumo Justificativa: O livedo reticular representa quadro dermatológico benigno, caracterizado por áreas isquêmicas permeadas por áreas eritematocianóticas em padrão rendilhado, pode ser transitório ou permanente e frequentemente é associado à exposição corporal ao frio. O vasoespasmo arterial cutâneo promove a isquemia e a dilatação venosa das áreas congestas e ocorre por hipóxia tecidual ou por disfunção autonômica. Os portadores da síndrome de Down, devido às suas peculiaridades fisiológicas, constituem uma representativa parcela daqueles que necessitam de atendimento odontológico sob anestesia geral e o livedo reticular tem incidência relatada de 8% a 12% em pacientes com síndrome de Down. Objetivos: Descrever quadro de livedo reticular fisiológico em paciente portador de síndrome de Down, com aparecimento durante o ato anestésico-cirúrgico. Relato do caso: Paciente de cinco anos, sexo feminino, síndrome de Down, admitida para tratamento odontológico sob anestesia geral balanceada, com emprego de sevoflurano, fentanil e atracúrio. Houve ocorrência de hipotermia transoperatória, a temperatura axilar atingiu 34,5 ºC após 30 minutos do início da anestesia. Ao término do procedimento, notaram-se lesões cutâneas vermelho-arroxeadas, intercaladas com áreas de palidez, exclusivamente na face ventromedial do antebraço direito, sem sinais sistêmicos sugestivos de reações alérgicas. O diagnóstico firmado foi de livedo reticular fisiológico. Houve esmaecimento total das lesões em cinco dias. Conclusão: O relato apresentado evidencia a necessidade de controle térmico de pacientes submetidos a anestesias, bem como registra manifestação de livedo reticular em consequência de hipotermia transoperatória.

Síndrome de Sneddon asociado a síndrome antifosfolipídico: descripciones clínicas y revisión de la literatura / Sneddon syndrome associated with antiphospholipid syndrome: clinical descriptions and a literature review

Resumen El síndrome de Sneddon es una rara vasculopatía no inflamatoria, obliterante, caracterizada por la asociación de eventos cardiovasculares (hipertensión arterial, claudicación intermitente y enfermedad coronaria), neurológicas (accidentes cerebrovasculares isquémicos, cefalea, vértigo y convulsiones) y livedo reticularis de tipo racemosa. Presentamos a una mujer que ingresa con un cuadro neurológico isquémico, hipertensión arterial, problemas vasculares y lesiones en piel. La biopsia de piel se catalogó como dermatitis perivascular superficial linfocitaria, sugestivo de lesión oclusiva.

Abstract Sneddon syndrome is a rare non-inflammatory obliterative vasculopathy, characterised by the association of cardiovascular (arterial hypertension, intermittent claudication, and coronary artery disease) and neurological events (ischaemic stroke, headache, dizziness and convulsions), and livedo reticularis/livedo racemosa. The case is presented of a woman admitted with an ischaemic neurological disease, hypertension, vascular problems, and skin lesions. The skin biopsy was classified as surface perivascular lymphocytic dermatitis, suggestive of occlusive lesion.

Vasculopatia livedóide: a propósito de um caso clínico / Livedoid vasculopathy: a clinical case proposition

A vasculopatia livedóide é uma dermatose crônica oclusiva dos vasos sanguíneos da derme, de caráter pauci-inflamatório ou não inflamatório, extremamente dolorosa. A etiologia não é totalmente esclarecida; estando associada a eventos pró-trombóticos. Caracterizada pela presença de lesões maculopapulares, eritêmato-purpúricas, nos membros inferiores, especialmente nos tornozelos e pés que ulceram gerando cicatrizes atróficas, denominadas atrofia branca. A histopatologia evidencia oclusão dos vasos da derme, devido à deposição de fibrina intravascular e presença de trombo intraluminal, além de hialinização segmentar e proliferação endotelial, há também, um infiltrado inflamatório perivascular discreto. Descrevemos um caso de vasculopatia livedóide evidenciando os achados histopatológicos encontrados na biópsia. (AU)

Livedoid vasculopathy is a chronic occlusive vasculopathy dermatosis, with a pauciinflammatory or noninflammatory characteristic, and is extremely painful. Its ethiology is not entirely known, being associated with pro-thrombotic events. Characterized by erythematous purpuric maculopapular lesions on the legs, especially on the ankles and feet, that ulcerates and heals with white atrophic scars, named atrophie blanche. Histopathology shows occlusion of dermal vases, segmental hyalinization and endothelial proliferation; there is also a discreet perivascular inflammatory infiltrate. We describe a case of livedoid vasculopathy showing the histopathological findings on the biopsy. (AU)

Clinical characteristics of systemic lupus erythematosus patients with cutaneous vasculitis / 中华皮肤科杂志

Objective To compare clinical and laboratory characteristics between systemic lupus erythematosus (SLE) patients with and without cutaneous vasculitis,and to investigate the correlation of cutaneous vasculitis with severe visceral involvement and laboratory biomarkers.Methods A total of 152 SLE patients with various skin manifestations were enrolled from Department of Dermatology of Huashan Hospital affiliated to Fudan University from July 2011 to October 2014.The clinical and laboratory data were collected and retrospectively analyzed.SLE patients with cutaneous vasculitis were divided into upper/lower extremity vasculitis group and livedo reticularis group.A logistic regression model was used to analyze the correlation between cutaneous vasculitis and various clinical and laboratory variables.Results Of 152 SLE patients,62 (41％) presented with cutaneous vasculitis,including 55 with upper/lower extremity vasculitis and 7 with livedo reticularis,and 90 (59％) did not have cutaneous vasculitis.Patients with upper/lower extremity vasculitis showed significantly younger age (30.54 ± 12.67 years vs.37.77 ± 12.17 years),and lower prevalence of aberrantly elevated 24-hour protein excretion (39.39％ vs.64.00％) and serum urea level (2.08％ vs.16.43％),but significantly higher percentage of females (98.18％ vs.84.44％),higher proportions of patients with abnormal brain MRI (37.5％ vs.12.19％),anemia (87.03％ vs.70.93％) and positive antiribosomal P protein antibodies (77.77％ vs.53.65％),and higher SLE disease activity index (SLEDAI) (14.71 ± 7.75 vs.10.68 ± 5.61) than those without vasculitis (all P ＜ 0.05).The proportion of patients with decreased C3 level did not differ between patients with upper/lower extremity vasculitis and those without cutaneous vasculitis (P =0.362),but was significantly lower in the patients with livedo reticularis than in those without cutaneous vasculitis (28.57％ vs.79.76％,P =0.008).However,no significant differences in the other variables were observed between patients with livedo reticularis and those without cutaneous vasculitis (all P ＞ 0.05).Additionally,body mass index (BMI),abnormal lung function and other laboratory variables all did not differ among patients with upper/lower extremity vasculitis,patients with livedo reticularis and patients without cutaneous vasculitis (all P ＞ 0.05).Logistic regression analysis revealed that after exclusion of potential effects of age and gender,cutaneous vasculitis was significantly positively correlated with abnormal brain MRI (OR =4.24,95％ CI:1.17-16.13,P =0.028),and positive anti-ribosomal P protein antibodies (OR =3.97,95％ CI:1.86-8.47,P =0.0004),but negatively correlated with abnormally elevated 24-hour protein excretion (OR =0.25,95％ CI:0.09-0.69,P =0.009).Furthermore,cutaneous vasculitis showed no significant associations with abnormal serum urea level (OR =0.12,95％ CI:0.01-1.06),decreased C3 level (OR =0.93,95％ CI:0.38-2.28),anemia (OR =1.38,95％ CI:0.56-3.40) or SLEDAI (OR =1.05,95％ CI:0.98-1.14).Conclusions Cutaneous vasculitis is closely associated with central nervous system damage and emergence of anti-ribosomal P protein antibodies,so SLE patients with cutaneous vasculitis should be closely monitored for central nervous system damage.SLE patients without cutaneous vasculitis are more liable to kidney injury,so they also need to be closely monitored.

Dermatological Manifestations of Postural Tachycardia Syndrome Are Common and Diverse

BACKGROUND AND PURPOSE: Postural tachycardia syndrome (POTS) is a syndrome of orthostatic intolerance in the setting of excessive tachycardia with orthostatic challenge, and these symptoms are relieved when recumbent. Apart from symptoms of orthostatic intolerance, there are many other comorbid conditions such as chronic headache, fibromyalgia, gastrointestinal disorders, and sleep disturbances. Dermatological manifestations of POTS are also common and range widely from livedo reticularis to Raynaud's phenomenon. METHODS: Questionnaires were distributed to 26 patients with POTS who presented to the neurology clinic. They were asked to report on various characteristics of dermatological symptoms, with their answers recorded on a Likert rating scale. Symptoms were considered positive if patients answered with "strongly agree" or "agree", and negative if they answered with "neutral", "strongly disagree", or "disagree". RESULTS: The most commonly reported symptom was rash (77%). Raynaud's phenomenon was reported by over half of the patients, and about a quarter of patients reported livedo reticularis. The rash was most commonly found on the arms, legs, and trunk. Some patients reported that the rash could spread, and was likely to be pruritic or painful. Very few reported worsening of symptoms on standing. CONCLUSIONS: The results suggest that dermatological manifestations in POTS vary but are highly prevalent, and are therefore of important diagnostic and therapeutic significance for physicians and patients alike to gain a better understanding thereof. Further research exploring the underlying pathophysiology, incidence, and treatment strategies is necessary.

Amantadine-induced livedo reticularis - Case report

AbstractLivedo reticularis is a spastic-anatomical condition of the small vessels which translates morphologically by a reticular pattern, interspersing cyanosis, pallor and erythema. The same can be congenital or acquired. Among the acquired, we highlight the physiological livedo reticularis and the idiopathic livedo by vasospasm; the latter configures the most common cause. The drug-induced type is less common. The drugs amantadine and norepinephrine are often implicated. Cyanosis is usually reversible if the causative factor is removed, however, with chronicity, the vessels may become permanently dilated and telangiectatic. We report a case of a patient diagnosed with Parkinson's disease with chronic livedo reticularis associated with the use of amantadine and improvement after discontinuation of the drug.

Livedoid vasculopathy associated with peripheral neuropathy: a report of two cases / Vasculopatia livedoide associada a neuropatia periferica: relato de dois casos

Livedoid vasculopathy (LV) is a chronic and recurrent disease consisting of livedo reticularis and symmetric ulcerations, primarily located on the lower extremities, which heal slowly and leave an atrophic white scar ("atrophie blanche"). Neurological involvment is rare and presumed to be secondary to the ischemia from vascular thrombosis of the vasa nervorum. Laboratory evaluation is needed to exclude secondary causes such as hyper-coagulable states, autoimmune disorders and neoplasms. We present two patients with a rare association of peripheral neuropathy and LV, thereby highlighting the importance of a multidisciplinary approach to reach the correct diagnosis. .

Vasculopatia livedoide é uma doença crônica e recorrente caracterizada por livedo reticular e úlceras simétricas nos membros inferiores, que cicatrizam e deixam uma cicatriz branca atrófica ("atrophie blanche"). Envolvimento neurológico é raro e está provavelmente associado a isquemia pela trombose dos vasa nervorum. Avaliação laboratorial é indicada com o intuito de excluir causas secundárias como estados de hipercoagulabilidade, doenças autoimunes e neoplasias. Apresentamos dois pacientes com uma rara associação de vasculopatia livedoide com neuropatia periférica, enfatizando a importância de uma abordagem multidisciplinar na busca do diagnóstico correto.

Síndrome de Sneddon: reporte de un caso / Sneddon syndrome: case report

El síndrome de Sneddon es una vasculitis sistémica, que se caracteriza por accidente cerebrovascular isquémico y livedo reticularis. Es una enfermedad rara, que se da principalmente en mujeres jóvenes. Presentamos el caso de una mujer de 20 años con el diagnóstico de Síndrome de Sneddon, tratada en forma oportuna y con una evolución favorable en el hospital Doctor Hernán Henríquez Aravena de Temuco (HHHA).

Sneddon's syndrome is a systemic vasculitis, characterized by ischemic stroke and livedo reticularis. It is a rare disease that occurs mainly in young women. We report the case of a 20 year old woman diagnosed with Sneddon syndrome, treated in time and with a favorable outcome at Dr. Hernán Henríquez Aravena Hospital (HHHA) of Temuco

Síndrome antifosfolípide e trombocitopenia na infância / Antiphospholipid syndrome and thrombocytopenia in childhood / Síndrome antifosfolípido y trombocitopenia en la infancia: relato de caso

OBJETIVO: Relatar o caso de uma criança com diagnóstico de síndrome do anticorpo antifosfolípide associada à trombocitopenia grave e realizar uma revisão de literatura sobre o assunto. DESCRIÇÃO DO CASO: Criança de nove anos e oito meses de idade com trombocitopenia grave associada a anticorpo anticardiolipina positivo. Os dados foram coletados por meio de anamnese, exame físico e exames complementares da paciente. O diagnóstico foi determinado de acordo com os critérios estabelecidos para a síndrome antifosfolípide, associados às manifestações mais comuns na faixa etária pediátrica: livedo reticular e trombocitopenia. COMENTÁRIOS: A síndrome do anticorpo antifosfolípide é uma doença incomum na população pediátrica e suas manifestações clínicas, com a redução do número de plaquetas, devem ser consideradas.

OBJECTIVE: To report the case of a child diagnosed with antiphospholipid syndrome associated with severe thrombocytopenia, and to review the literature on the subject. CASE DESCRIPTION: Child aged nine years and eight months old with severe thrombocytopenia associated with a positive anticardiolipin antibody. Data were collected by clinical history, physical examination, and laboratorial exams. Diagnosis was confirmed according to criteria established for the antiophospholipid syndrome, associated with the presence of the most common manifestations of the syndrome in children: livedo reticularis and thrombocytopenia. COMMENTS: The antiphospholipid syndrome is an uncommon pediatric disease, and clinical manifestations such as decreased platelet number should be considered.

OBJETIVO: Relatar el caso de un niño con diagnóstico de síndrome del anticuerpo antifosfolípido asociado a trombocitopenia grave y realizar una revisión de literatura sobre el tema. DESCRIPCIÓN DEL CASO: Niño de nueve años y ocho meses de edad, con trombocitopenia grave asociada a anticuerpo anticardiolipina positivo. Los datos fueron recogidos por medio de historia, examen físico y exámenes complementarios de la paciente internada en un hospital de Curitiba, en Paraná (Brasil). El diagnóstico fue determinado conforme a los criterios establecidos para el síndrome antifosfolípido, asociados a las manifestaciones más comunes en la franja de edad pediátrica: livedo recticular y trombocitopenia. COMENTARIOS: El síndrome del anticuerpo antifosfolípido es una enfermedad poco común en la población pediátrica, y su manifestación con reducción del número de plaquetas debe ser considerada.

Sneddon's syndrome: case report and review of its relationship with antiphospholipid syndrome / Síndrome de Sneddon: relato de caso e revisão sobre a relação com a síndrome do anticorpo antifosfolipídio

The Sneddon's syndrome is a rare disorder characterized by the occurrence of cerebrovascular disease associated with livedo reticularis. The antiphospholipid syndrome is the most frequent type of acquired thrombophilia, defined by the occurrence of thrombosis or pregnancy morbidity in the presence of persistently positive antiphospholipid antibodies. Approximately 80% of Sneddon's syndrome patients have an antiphospholipid antibody marker. These antibodies may play a pathogenetic role in some cases of Sneddon's syndrome, and many authors consider these two syndromes as the same entity. Although clinical features of antiphospholipid syndrome and Sneddon's syndrome may overlap, there is a distinction between clinical and laboratory evidence suggesting that these two entities are different diseases. A recent finding of coagulopathies, including elevated levels of coagulation factor VII, decreased levels of protein S, and activated protein C in Sneddon's syndrome patients suggested a possible biological link between the vasculopathy and a primary coagulopathy. Moreover, the clinical course seems to be progressive in Sneddon's syndrome patients and includes increase of disability and cognitive deterioration, more arterial involvement, and the antiphospholipid syndrome shows a more benign course. Both syndromes share clinical and laboratory features, and whether Sneddon's syndrome represents a spectrum of antiphospholipid syndrome remains unclear. Sneddon's syndrome patients have a worse prognosis and may represent a subgroup of patients who demands more rigorous follow-up. It is important to recognize the Sneddon's syndrome, particularly because stroke episodes may be prevented through appropriate treatment.

A síndrome de Sneddon é um distúrbio raro caracterizado pela ocorrência de doença cerebrovascular associada a livedo reticular. A síndrome do anticorpo antifosfolipídio é o tipo mais frequente de trombofilia, definida pela ocorrência de trombose ou morbidade gestacional na presença de anticorpos antifosfolípides persistentemente positivos. Aproximadamente 80% dos pacientes com síndrome de Sneddon apresentam um marcador de anticorpo antifosfolipídio. Esses anticorpos podem exercer um papel fisiopatológico em alguns casos de síndrome de Sneddon, e muitos autores consideram essa síndrome e a síndrome do anticorpo antifosfolipídio a mesma entidade. Apesar de os quadros clínicos das suas síndromes poderem se sobrepor, há evidência clínica e laboratorial distintiva, sugerindo que as duas entidades são doenças diferentes. Um achado recente de coagulopatia, incluindo níveis elevados do fator VII de coagulação, diminuição dos níveis da proteína S, e proteína C ativada em pacientes com síndrome de Sneddon, sugeriu uma possível ligação biológica entre a vasculopatia e coagulopatia primária. Além disso, o curso clínico pareceu ser progressivo em pacientes com síndrome de Sneddon, visto que há aumento de incapacidade e deterioração cognitiva, além de maior envolvimento arterial, enquanto a síndrome do anticorpo antifosfolipídio apresenta um curso mais benigno. Ambas as síndromes compartilham características clínicas e laboratoriais; até qual ponto a síndrome de Sneddon representa um espectro da síndrome do anticorpo antifosfolipídio permanece desconhecido. Os pacientes com a primeira síndrome apresentam pior prognóstico e podem representar um subgrupo de pacientes que requer um seguimento mais rigoroso. É importante reconhecer a síndrome de Sneddon já que os episódios de acidente vascular cerebral podem ser prevenidos com a terapia apropriada.

Case for diagnosis / Caso para diagnóstico

We report the case of a 58-year-old white female with Parkinson's disease. She evolved to an extensive livedo reticularis in the limbs and abdomen after commencing treatment with amantadine. We discuss the diagnostic approach to livedo reticularis and its differential diagnoses, emphasizing that the drug etiology must be considered when investigating livedo reticularis.

Descreve-se caso clínico de paciente feminina adulta, portadora de doença de Parkinson em uso de amantadina que desenvolveu extenso quadro de livedo reticular nos membros e abdome após o início do medicamento. Discutem-se a semiotécnica diagnóstica do livedo reticular e seus diferenciais. Os autores salientam que a etiologia medicamentosa deva ser considerada no diagnóstico dos livedos reticulares.

Livedo reticular associado com Anemia Hemolítica Autoimune: remissão prolongada induzida pelo Transplante de Células-Tronco do Sangue Periférico com recaída após 10 anos e restauração dos níveis de hemoglobina por rituximabe / Livedo reticularis associated with autoimmune hemolytic anemia: prolonged remission induced by peripheral blood stem cell transplantation relapse after 10 years and restoration of hemoglobin levels by rituximab

A anemia hemolítica autoimune (AHAI) é uma doença na qual são produzidos anticorpos diretamente contra as glicoproteínas adsorvidas na superfície da membrana dos eritrócitos. Algumas medicações e outras associações têm sido implicadas. Descrevemos e discutimos um caso de livedo reticular associado à AHAI tratado com transplante de células-tronco de sangue periférico (TCTSP) e que entrou em total remissão por 10 anos. Após esse período, a paciente apresentou recaída, foi tratada com anticorpo anti-CD20 (rituximabe), e atualmente encontra-se em total remissão. O papel do TCTSP e o uso de rituximabe no tratamento de AHAI serão discutidos neste relato de caso.

Autoimmune hemolytic anemia (AIHA) is a disease where patients produce antibodies against erythrocytes directed towards membrane glycoproteins adsorbed onto the erythrocyte surface. Drugs and other associations have been implicated. It is described and discussed a case of livedo reticularis associated with AIHA treated with peripheral blood stem cell transplantation (PBSCT) that went into full remission for 10 years. After that period the patient relapsed and was treated with antibody anti-CD20, rituximab, and is now in full remission. The role of PBSCT and rituximab in the treatment of AIHA will be discussed.