Retinal abnormalities in a patient with cutis marmorata telangiectatica congenita.

Cutis marmorata telangiectatica congenita is a rare congenital vascular malformation characterised by cutaneous vascular abnormalities, typically diagnosed at birth or in the early postnatal period. Although typically benign, this disease is associated with other systemic abnormalities, including rare ocular alterations, such as congenital glaucoma, cataracts and retinopathy.This manuscript describes a female infant, who presented with generalised livedo reticularis, a band of alopecia and cutaneous atrophy in the temporal region above the coronal suture. The patient was diagnosed with cutis marmorata telangiectatica congenita by a paediatrician, and an ophthalmological evaluation was requested. A funduscopy examination in both eyes showed temporal and superior retina with avascular areas with new vessels, venous dilations and shunts, and no retinal detachments. Given these findings, we performed retinal photocoagulation laser treatment with excellent results.This case report highlights the importance of early ophthalmological evaluation of children with this disease to prevent secondary complications, such as vitreous haemorrhage and tractional retinal detachment.

Vasculitic rash and cerebral emboli in an adolescent with left atrial myxoma.

A previously healthy woman in late adolescence presented to the emergency department with stroke-like symptoms following a two-month history of bilateral foot pain and oedema, accompanied by a macular rash and progressive lower extremity weakness. On further investigation, she was found to have multiple cerebral emboli and a left atrial myxoma fixed to the interatrial septum. The patient subsequently underwent urgent surgical excision of the myxoma. On follow-up, her cutaneous and neurological symptoms were significantly improved. This case suggests that, in the presence of a vasculitic rash without evident or obvious cause, cardiac myxoma should be included in the differential diagnosis.

[Secondary vasculitides]. / Sekundäre Vaskulitiden.

Vasculitides that occur in association with underlying primary diseases are called secondary vasculitides. In the diverse differential diagnostics of vasculitides, a large variety of secondary vasculitides have to be considered. Secondary vasculitides cover the full spectrum of vasculitides, presenting in manifold clinical manifestations. This article provides an overview of systemic diseases and etiological factors, such as infections, drugs, and malignancies, which can be associated with vasculitides. The possible associations with infectious agents are too numerous to be comprehensively covered and are discussed in an exemplary fashion and with a western European focus. Especially in atypical and refractory disease courses, a secondary vasculitis should be considered. In light of the diversity of differential diagnoses and the particular challenges posed by secondary vasculitides, interdisciplinary collaboration is the key for an accurate and early diagnosis as well as for successful treatment management. Treatment of the primary disease should always be prioritized and, if a drug-induced vasculitis is suspected, immediate cessation of the culprit drug is mandatory.

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)

Características de las amputaciones mayores en pacientes con isquemia arterial aguda trombótica de miembros inferiores / Characteristics of major amputations in patients with acute thrombotic arterial ischemia of the lower limbs

Introducción: las amputaciones mayores de causa vascular justifican el 60 por ciento de las amputaciones que se realizan en los servicios de cirugía vascular. El alto grado de complicaciones aterotrombóticas, entre ellas, la trombosis, gangrena e infección, que presentan los pacientes diabéticos y no diabéticos, como consecuencia de estos eventos vasculares, conllevan a que la amputación mayor continúa siendo parte de la práctica quirúrgica de los cirujanos vasculares. Objetivo: describir las características de las amputaciones mayores de miembros inferiores en pacientes con isquemia arterial aguda trombótica.Métodos: estudio descriptivo, retrospectivo, de corte transversal, en 150 pacientes que habían ingresado en los servicios de Arteriología y Angiopatía Diabética del Instituto Nacional de Angiología y Cirugía Vascular durante un período de cinco años. A 71 de ellos se les realizó una amputación mayor. Resultados: se encontró un predominio de pacientes del sexo masculino (n= 44; 61,97 por ciento); e independientemente del sexo, una mayor frecuencia de enfermos con más de 60 años. Hubo superioridad de pacientes no diabéticos (67,6 por ciento). Al analizar el comportamiento del nivel de amputación mayor se halló que fue en ellos donde ocurrió la mayor práctica de amputaciones supracondíleas (69,6 por ciento). Solo se realizaron dos amputaciones infracondíleas y fue en los no diabéticos. Conclusiones: el número de amputaciones mayores por causa de isquemia arterial aguda trombótica de miembros inferiores aumenta con la edad, con predominio del sexo masculino en pacientes no diabéticos(AU)

Introduction: major amputations of vascular origin accounts for 60 percent of the amputations that are performed in the vascular surgery services. The high rate of atherothrombotic complications (thrombosis, gangrene and infection) occurring in diabetic and non diabetic patients as a result of these events discloses that major amputation remains part of the surgical practice of the vascular surgeons. Objective: to describe the characteristics of major amputations of lower limbs in patients suffering acute thrombotic arterial ischemia.Methods: retrospective, descriptive and cross-sectional study of 150 patients who had been admitted to the arteriology and diabetic angiopathy service of the National Institute of Angiology and Vascular Surgery in a 5 year-period. Seventy one of them had undergone major amputation. Results: Males (44; 61.97 percent) and patients aged over 60 years, regardless of sex, predominated. It was observed that non-diabetic patients (67. 6 percent) prevailed. On analyzing the behaviour of the rate of major amputation, it was found that the highest number of suprachondylear amputationms was practiced in this group of patients (69.6 percent). Just two infrachondylear amputations were performed in non-diabetic patients.Conclusions: the number of major amputations caused by acute thrombotic arterial ischemia of lower limbs increases with the age, being predominant in non-diabetic male patients(AU)

Recién nacido con lesiones cutáneas reticuladas / Newborn with reticular cutaneous lesions

Introducción. Las lesiones cutáneas aparecidas en los primeros días de vida o desde el nacimiento no son siempre de fácil diagnóstico y la mayoría de las veces la orientación clínica o la biopsia serán la forma de establecer el diagnóstico. El cutis marmorata telangiectásico congénito es una infrecuente malformación vascular cutánea que en más del 90 por ciento de los casos está presente al nacimiento y cuyo pronóstico es bueno. Observación clínica. Lactante que desde el primer día de vida presenta unas lesiones reticuladas en una extremidad inferior y en la zona sacroperineal. Se establece el diagnóstico diferencial con posibles entidades de aspecto similar y se realiza la orientación diagnóstica de forma clínica. Comentarios. Se dan las bases para el diagnóstico diferencial con determinadas entidades que en alguna forma tienen similitud cutánea y se comenta la posibilidad de asociación con alguna malformación congénita así como la posible evolución de la enfermedad(AU)

Introduction. Skin lesions that are present at birth or develop during the first days of life are not always easy to diagnose; a careful clinical evaluation, which may include a biopsy, is needed. The cutis marmorata telangiectatica congenita is a rare cutaneous vascular malformation that is present at birth in more than 90% of the cases and that has an excellent prognosis. Clinical Observation. Newborn infant who presented with reticular skin lesions in a lower extremity and in the sacral and perineal areas. A clinical diagnosis of cutis marmorata telangiectatica congenita was made after considering other entities with similar presentation. Comments. The differential diagnosis of this entity, its possible associations with congenital abnormalities, and its clinical course are discussed(AU)

Panarteritis nudosa cutánea en la infancia / Childhood cutaneous polyarteritis nodosa

La panarteritis nudosa cutánea es una forma poco frecuente de vasculitis necrosante de arterias de pequeño y mediano calibre, de curso crónico y recurrente, que puede acompañarse defiebre, artralgias, mialgias y neuropatía, pero sin compromiso sistémico. Se presenta un caso de panarteritis nudosa cutánea.

Panarteritis nudosa cutánea en la infancia / Childhood cutaneous polyarteritis nodosa

La panarteritis nudosa cutánea es una forma poco frecuente de vasculitis necrosante de arterias de pequeño y mediano calibre, de curso crónico y recurrente, que puede acompañarse defiebre, artralgias, mialgias y neuropatía, pero sin compromiso sistémico. Se presenta un caso de panarteritis nudosa cutánea.(AU)

Livedo racemosa as a marker of increased risk of recurrent thrombosis in patients with negative anti-phospholipid antibodies

Background and objective: Livedo reticularis racemosa and cerebrovascular lesions characterize Sneddon's syndrome. We report 23 patients with livedo racemosa and describe the association with thrombotic events. Our objective was to determine whether livedo racemosa may be an independent clinical marker for the development of thrombotic events in patients who test negative for anti-phospholipid antibodies. Methods: Twenty-three patients with widespread livedo racemosa were studied. None of the patients were positive for anti-phospholipid antibodies. The clinical protocol included a register of thrombotic events, fetal death or miscarriages, hypertension, and valvular heart disease. Cerebral MRI and echocardiography were systematically performed in all patients. Results: Nineteen patients (82.60%) had thrombotic events. Fifteen (65.21%) had arterial thrombosis and eleven (47.82%) presented venous occlusions. Seven patients (30.43%) had both arterial and venous thrombosis. Fetal losses were recorded in seven cases (30.43%), with a total number of 33; five patients had 3 or more fetal losses. Eleven out of 23 patients (47.82%) had valvular heart disease. Arterial hypertension was detected in 16 (69.56%) patients. Four patients did not have thrombotic events but had other clinical manifestations. After anti-coagulation therapy was withdrawn, a new thrombotic event was observed in 9 out of the 14 treated patients (64.28%).Conclusions: Livedo racemosa seems to be a good clinical marker for the detection of hypercoagulable states even in the absence of anti-phospholipid antibodies or other known biologic markers of thrombosis. Long-term anti-coagulation is probably warranted in patients with livedo racemosa and a previous thrombotic event (AU)

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A propósito de un signo cutáneo de vasculopatía sistémica / Considerations on a cutaneous manifestation of systemic vasculopathy

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Cutis marmorata telangiectásica congénita. Revisión de 33 casos / Cutis marmorata telangiectasia congénita. Review of 33 cases

Introducción: La cutis marmorata telangiectásica congénita (CMTC) es una infrecuente malformación vascular cutánea que se caracteriza por la presencia de un patrón localizado o generalizado, casi siempre asimétrico, de máculas reticuladas eritematosas o violáceas, presentes desde el nacimiento o poco después de éste. Pacientes y método: Estudio retrospectivo de 33 casos de CMTC diagnosticados entre 1994 y 2007 en la consulta de dermatología pediátrica de nuestro hospital, recogiéndose datos clínicos y evolutivos, y en algunos casos, otros exámenes complementarios. Resultados: El 51,5 % de los pacientes fueron mujeres. En el 87,9% de los casos las lesiones se observaron en el momento del nacimiento. Todos los casos fueron esporádicos. En el 72,7 % la distribución de las lesiones fue localizada, sobre todo afectando a los miembros inferiores. Se apreciaron anomalías asociadas en el 60,6 % de los casos, entre las que destacaban las alteraciones cutáneas (14 casos); entre las alteraciones extracutáneas, presentes en 11 pacientes, destacaron los trastornos tróficos de los miembros. En ningún caso se apreciaron alteraciones oftalmológicas o neurológicas. La mediana del tiempo de seguimiento fue de 14 meses. El 45 % de los pacientes mejoró y en un caso se objetivó la total resolución de las lesiones cutáneas. Conclusiones: La CMTC es una enfermedad malformativa con frecuentes anomalías asociadas. Es conveniente realizar una adecuada valoración y seguimiento de los pacientes. Consideramos necesaria la realización de una historia clínica completa en la que se incluya una exhaustiva exploración. Si la enfermedad afectase a la cabeza deberían realizarse exploraciones neurológica y oftalmológica. Generalmente, el pronóstico es bueno, y las lesiones cutáneas tienden a mejorar o a desaparecer en la mayoría de los casos (AU)

Introduction: Cutis marmorata telangiectatica congenita (CMTC) is an uncommon congenital vascular malformation characterized by the presence of a localized or generalized pattern, frequently asymmetrical, with reticulated, erythematous or violaceous macules, present at birth or shortly thereafter. Patients and method: Retrospective study of 33 cases of CMTC diagnosed between 1994 and 2007 in our hospital. Clinical and follow-up data were recorded in all cases. In some patients additional tests were performed. Results: Most of the patients (51.5 %) were female. In 87.9 % of the cases lesions were observed at birth. All the cases were sporadic. CMTC was localized in 72.7% of the patients, being distributed mainly over the lower limbs. Associated anomalies were noted in 60.6 %, with predominance of skin alterations (14 cases). Extracutaneous anomalies were present in 11 patients, most commonly atrophy and hypertrophy in the involved limbs. In none of the cases were there ocular or neurological manifestations. The average follow-up time was 14 months. Improvement of the lesions was observed in 45 %, and in one case there was complete resolution. Conclusions: CMTC is a malformative disease which is frequently associated with other abnormalities. An appropriate assessment and follow-up of these patients is advisable. Performing a thorough medical history, including full physical examination is necessary. If the head is affected, ocular and neurological examination should be performed. The prognosis is generally good, with a tendency to improvement or disappearance in most cases (AU)

Livedo reticularis de las piernas: metodología de diagnóstico y tratamiento / Diagnosis and treatment of livedo reticularis on the legs

El término livedo reticularis describe un retículo cutáneo de coloración rojo-violácea que afecta preferentemente a las extremidades. Su origen es la interrupción del flujo sanguíneo en las arteriolas dérmicas, ya sea por espasmo, inflamación u obstrucción intravascular, y se asocia a entidades de diversa etiología y gravedad. Para establecer la causa de una livedo reticularis es fundamental conocer la evolución del cuadro (si la livedo reticularis es o bien crónica o bien aguda o fulminante), la presencia de otros signos cutáneos, como nódulos, púrpura retiforme o necrosis, y la posible asociación con síntomas generales y/o datos analíticos que apunten hacia un determinado proceso sistémico. El objetivo de esta revisión es proporcionar un método diagnóstico y terapéutico para el abordaje de la livedo reticularis (AU)

The term livedo reticularis refers to a reddish-violet reticular discoloration of the skin that mainly affects the limbs. It is caused by an interruption of blood flow in the dermal arteries, either due to spasm, inflammation, or vascular obstruction, and is associated with diseases of varying etiology and severity. To establish the cause of livedo reticularis, it is essential to determine its course (chronic, acute, or fulminant), the presence of other cutaneous signs such as nodules, retiform purpura or necrosis, and the possible association of general symptoms or laboratory findings that suggest a particular systemic process. The aim of this review is to describe the diagnosis and treatment of the disease (AU)

Livedo reticularis y vasculopatía trombótica / Livedo reticularis and thrombosis

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Síndrome de Sneddon y pioderma gangrenoso / Sneddon’s syndrome and pyoderma gangrenosum

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Cutaneous Polyarteritis Nodosa Presented with Digital Gangrene: A Case Report

Cutaneous polyarteritis nodosa (CPAN) is an uncommon form of vasculitis involving small and medium sized arteries of unknown etiology. The disease can be differentiated from polyarteritis nodosa by its limitation to the skin and lack of progression to visceral involvement. The characteristic manifestations are subcutaneous nodule, livedo reticularis, and ulceration, mostly localized on the lower extremity. Arthralgia, myalgia, peripheral neuropathy, and constitutional symptoms such as fever and malaise may also be present. We describe a 34-yr-old woman presented with severe ischemic change of the fingertip and subcutaneous nodules without systemic manifestations as an unusual initial manifestation of CPAN. Therapy with corticosteroid and alprostadil induce a moderate improvement of skin lesions. However, necrosis of the finger got worse and the finger was amputated.