Case for diagnosis. Cutaneous small vessel vasculitis (anti-proteinase 3 positive), fever, hemoptysis, and lung cavitation in an adult

Abstract Small vessel vasculitis with anti-proteinase antibodies 3 is an atypical clinical presentation of tuberculosis. The authors present the case of a 47-year-old male patient, with palpable purpura and palmoplantar hemorrhagic blisters, with subsequent dissemination. He presented severe pulmonary symptoms with cavitation, fever, hemoptysis, and high levels of anti-proteinase 3. Histopathological assessment of the skin revealed small vessel vasculitis; pulmonary histopathology showed granulomas with caseation. Bronchoalveolar lavage was positive for alcohol-acid-fast bacilli. In countries with a high prevalence of tuberculosis, the presence of autoantibodies in a patient with vasculitis, fever, and pulmonary cavitation requires investigation of infectious causes.

Ophthalmologic alterations in cutis marmorata telangiectatica congenita: a series of cases / Alterações oftalmológicas na cutis marmorata telangiectásica congênita: uma série de casos

ABSTRACT Cutis marmorata telangiectasia congenita is a rare cutaneous vascular disorder that may be associated with different systemic manifestations like body asymmetry, cutaneous, ophthalmologic, vascular, and neurological manifestations. We describe ophthalmologic alterations found in three patients with cutis marmorata telangiectatica congenita highlighting the rare retinal manifestations.

RESUMO A Cutis marmorata telangiectasica congênita é uma desordem vascular cutânea rara que pode estar associada a diferentes manifestações sistêmicas, como assimetria corporal, alterações cutâneas, oftalmológicas, vasculares e neurológicas. Descrevemos alterações oftalmológicas encontradas em três pacientes com cutis marmorata telangiectasia congênita, destacando as raras manifestações retinianas.

Update on vasculitis: an overview and dermatological clues for clinical and histopathological diagnosis - part I

Abstract The term vasculitis refers to the inflammation of vessel walls. It may range in severity from a self-limited disorder in one single organ to a life-threatening disease due to multiple organ failure. It has many causes, although they result in only a few histological patterns of vascular inflammation. Vessels of any type and in any organ can be affected, a fact that results in a broad variety of signs and symptoms. Different vasculitides with indistinguishable clinical presentations have quite different prognosis and treatments. This condition presents many challenges to physicians in terms of classification, diagnosis, appropriate laboratory workup, and treatment. Moreover, it compels a careful follow-up. This article reviews the Chapel-Hill 2012 classification, etiology, recent insights in pathophysiology, some important dermatological clues for the diagnosis and summarizes treatment of some of these complex vasculitis syndromes.

Intravascular histiocytosis: case report of a rare disease probably associated with silicone breast implant

Abstract Intravascular histiocytosis is a rare condition characterized by the aggregate of histiocytes within dilated dermal vessels. The diagnosis is mainly histophatological and immunohistochemical. We describe a case of a 55 year-old female patient presenting erythematous/purple patches on the breasts, back and limbs. She previously presented ductal carcinoma in the right breast in 2006 which was treated with mastectomy and proceeded to silicone breast implant in 2009. Clinical hypothesis was telangiectatic metastatic carcinoma. Histopathology showed vascular ectasia, thrombosis and recanalization of upper dermis small vessels. On immunohistochemistry, intravascular cells were CD 68+ and negative for estrogen and progesterone receptors, CK7, EMA and AE1/AE3 and endothelial cells were CD64+, leading to the diagnosis of intravascular histiocytosis.

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.

Relato de Caso: Síndrome de Sneddon por Anticorposantifosfolipides / Sneddon's Syndrome by Antiphospholipid Antibodies: Case Report

Introdução: A Síndrome de Sneddon, descrita em 1965, caracteriza-se pela associação de eventos isquêmicos cerebrovasculares com livedo reticular. É rara e tem predomínio em mulheres jovens. Seus sintomas variam de ataques isquêmicos transitórios a acidentes vasculares cerebrais isquêmicos, causado pela obliteração de artérias de pequeno a médio porte, levando a alterações patológicas como a inflamação endotelial, posterior proliferação subintimal e então fibrose. A evolução é lenta e progressiva, podendo levar ao óbito ou incapacidade. Objetivos: descrever o caso de uma paciente com Síndrome de Sneddon que acompanha no Hospital Universitário Evangélico Mackenzie e revisar a literatura atual. Descrição do Caso: mulher, 37 anos conta história de quatro episódios de AVE isquêmico e um de AVE hemorrágico, tendo o primeiro sido aos 22 anos e resultando em afasia, convulsões e hemiparesia a D. Na investigação detectou-se hipertensão arterial, livedo reticularis, nódulos subcutâneos e anticardiolipinas positivas em altos títulos (acima de 100 U/mL), além de plaquetopenia (n=70.000/mm3). Uma ecocardiografia mostrou insuficiência mitral com vegetações em válvulas. A paciente tinha, também, alteração da função renal e uma biópsia mostrou lesão intersticial não imune. Com diagnóstico de síndrome de Sneddon secundária à sindrome do anticorpo antifosfolipide, (SAF) a paciente foi anticoagulada com cumarinicos e sua hipertensão foi controlada com losartana. A investigação para lúpus eritematoso sistêmico subjacente foi negativa Conclusão: SAF é uma das causas da Síndrome de Sneddon e deve ser lembrada toda vez que uma paciente jovem se apresentar com AVEs isquêmicos.

Background: Sneddon's syndrome, described in 1965, is characterized by the association of cerebrovascular ischemic events with livedo reticularis. It is rare and predominates in young women. Its symptoms range from transient ischemic attacks to ischemic strokes, caused by the obliteration of small to medium-sized arteries, leading to pathological changes such as endothelial inflammation, subsequent subintimal proliferation and then fibrosis. The evolution is slow and progressive, leading to death or disability. Aim: Describe the case of a patient with Sneddon Syndrome who is being followed up at Hospital Universitário Evangélico Mackenzie and review the current literature. Case description: A 37-year-old woman tells the story of four episodes of ischemic stroke and one of hemorrhagic stroke, the first being at 22 years of age and resulting in aphasia, seizures and hemiparesis to D. In the investigation, arterial hypertension, livedo reticularis, subcutaneous nodules and positive anticardiolipins in high titers (above 100 U / mL), in addition to thrombocytopenia (n = 70,000 / mm3). An echocardiography showed mitral insufficiency with valve vegetations. The patient also had impaired renal function and a biopsy showed a non-immune interstitial lesion. With a diagnosis of Sneddon's syndrome secondary to the antiphospholipid antibody syndrome (APS), the patient was anticoagulated with cumadinicos and her hypertension was controlled with losartan. The investigation for underlying systemic lupus erythematosus was negative. Conclusion: APS is one of the causes of Sneddon Syndrome and must be remembered whenever a young patient presents with ischemic strokes.

Efectividad del propranolol en el tratamiento de malformaciones vasculares cutáneas / Effectiveness of propranolol in treatment of cutaneous vascular malformations

Se realizó un estudio cuasi-experimental de series temporales para evaluar la efectividad del propranolol en el tratamiento de malformaciones vasculares cutáneas en 48 pacientes que asistieron a la consulta del Servicio de Dermatología del Hospital Central Universitario Dr. Antonio María Pineda durante el período febrero-julio 2018. Los resultados muestran que existen diferencias estadísticamente significativas (p <0.05; p0,0001) antes y después del primer mes de tratamiento con propranolol, las cuales se mantiene hasta los seis meses, con respecto al tamaño, color, consistencia y temperatura. Se espera que los resultados sirvan para proponer el uso de propranolol como una opción terapéutica no invasiva en el tratamiento de las malformaciones vasculares cutáneas(AU)

A quasi-experimental study of time series was carried out to evaluate the effectiveness of propranolol in the treatment of cutaneous vascular malformations in 48 patients attending the Dermatology Service of the Hospital Central Universitario Dr. Antonio Maria Pineda during the period February - July 2018. The results show that there are statistically significant differences (p <0.05; p0,0001) before and after treatment with propranolol starting one month post-treatment which are kept until six months, related to size, color, consistency and temperature of lesions. We hope that these results will encourage the use of propranolol as a non-invasive therapeutic option in the treatment of cutaneous vascular malformations(AU)

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)

Cutaneous collagenous vasculopathy: light and transmission electron microscopy

Abstract Cutaneous collagenous vasculopathy is a rare acquired idiopathic microangiopathy characterized by progressive development of diffuse asymptomatic telangiectasias and histologically by accumulation of collagen type IV around the affected vessels. It is diagnosed by its clinical history, confirmed by light microscopy with collagen-specific immunostaining. We report a case of a patient with extensive acquired telangiectasias on the left arm, clinically resembling unilateral nevoid telangiectasia. Dilated blood vessels with thickened walls were observed in the dermis. Immunohistochemistry with collagen IV antibodies revealed marked collagen deposition around the vessels, confirming the diagnosis. Transmission electron microscopy observed duplicate and triplicate vascular basal membrane associated with deposition of amorphous material around the membranes.

Reporte de caso de granuloma facial, una vasculitis cutánea infrecuente / A case report of granuloma faciale, an uncommon cutaneous vasculitis

El granuloma facial es una dermatosis benigna poco frecuente de etiología desconocida, generalmente asintomática, caracterizada por inflamación crónica y localizada habitualmente en zonas fotoexpuestas de la cara, con un patrón histológico característico. Si bien la respuesta al tratamiento es variable, existen múltiples opciones terapéuticas que han reportado ser efectivas en algunos pacientes. Entre estas alternativas se incluyen tratamientos sistémicos con corticoides y dapsona o tratamientos intralesionales con corticoides, crioterapia e inhibidores de calcineurina tópicos. Describimos el caso de un paciente adulto con una placa eritemato violácea asintomática en mejilla derecha, de crecimiento lentamente progresivo de dos años de evolución, clínica e histopatológicamente compatible con granuloma facial y respuesta favorable a corticoides intralesionales.

Granuloma faciale is an uncommon benign dermatosis, with unknown etiology, usually asymptomatic, characterized by chronic inflammation localized in sun-exposed areas of the face with a characteristic histological pattern. Although response to treatment is variable, there are multiple therapeutic alternatives that have been reported to be effective in some patients, including systemic treatments with steroids and dapsone or also topical treatments like intralesional corticosteroid, cryotherapy and calcineurin inhibitors, such as tacrolimus. We present the case of an adult patient with an asymptomatic erythematous-violet plaque on the right cheek, with progressive slow growth over two years, clinically and histologically and pathologically compatible with a facial granuloma. The patient responded well to intralesional corticosteroids.

Pigmented purpura and cutaneous vascular occlusion syndromes

Abstract: Purpura is defined as a visible hemorrhage in the skin or mucosa, which is not evanescent upon pressure. Proper classification allows a better patient approach due to its multiple diagnoses. Purpuras can be categorized by size, morphology, and other characteristics. The course varies according to the etiology, as do the diagnostic approach and treatment. This review discusses pigmented purpuras and some cutaneous vascular occlusion syndromes.

Piel marmórea telangiectasica congenita y sindactilia en un recién nacido prematuro: A proposito de un caso / Congenital cutis marmorata telangiectatica and syndactyly in a preterm: Case report

La piel marmórea telangiectásica congenita (cutis marmorata telangiectatica congenita, CMTC) es una anomalía vascular congenita rara, a menudo benigna, localizada o generalizada, de etiología desconocida. Se caracteriza por piel marmórea persistente, telangiectasia y flebectasia. Podrían presentarse manifestaciones extracutáneas asociadas con la CMTC en el 18,8-70% de los casos. El diagnóstico de este trastorno se basa en los hallazgos clínicos. El pronóstico es bueno y suele mejorar dentro de los dos años de vida. En este artículo presentamos el caso de un varón recien nacido con CMTC en la piel de todas las extremidades, el tronco y el rostro, y una anomalía asociada, que incluía sindactilia. Presentamos este caso debido a su rareza.

Cutis marmorata telangiectatica congenita (CMTC) is a rare, commonly benign, congenital, localized or generalized vascular anomaly of unknown aetiology. It is characterized by persistent cutis marmorata, telangiectasia and phlebectasia. Extracutaneous findings may be associated with CMTC in 18.8-70% of the cases. Diagnosis of the disorder is based on the clinical findings. The prognosis is good and improvement is observed within 2 years after birth. Herein, we report a case of a male neonate with CMTC presented on the skin of all his limbs, trunk and face, and an associated anomaly including syndactyly. We present this case because of its rarity.

Vascular structures in dermoscopy

AbstractDermoscopy is an aiding method in the visualization of the epidermis and dermis. It is usually used to diagnose melanocytic lesions. In recent years, dermoscopy has increasingly been used to diagnose non-melanocytic lesions. Certain vascular structures, their patterns of arrangement and additional criteria may demonstrate lesion-specific characteristics. In this review, vascular structures and their arrangements are discussed separately in the light of conflicting views and an overview of recent literature.

The role of KIR2DL3/HLA-C*0802 in Brazilian patients with rheumatoid vasculitis

OBJECTIVES: Rheumatoid arthritis is a polygenically controlled systemic autoimmune disease. Rheumatoid vasculitis is an important extra-articular phenotype of rheumatoid arthritis that can result in deep cutaneous ulcers. The objective of this study was to establish a correlation between the frequency of major histocompatibility complex class I/II alleles and killer immunoglobulin-like receptor genotypes in patients with cutaneous rheumatoid vasculitis. METHODS: Using the Scott & Bacon 1984 criteria to diagnose rheumatoid vasculitis and after excluding any other causes such as diabetes, atherosclerosis, adverse drug reactions, infection, and smoking, patients who met the criteria were selected. All of the selected rheumatoid vasculitis patients presented deep cutaneous ulcers. Identification of the major histocompatibility complex class I/II and killer immunoglobulin-like receptor genotypes was performed by polymerase chain reaction assays of samples collected from the 23 rheumatoid vasculitis patients as well as from 80 controls (40 non-rheumatoid vasculitis RA control patients and 40 healthy volunteers). RESULTS: An association between the presence of the HLA-DRB1*1402 and HLA-DRB1*0101 alleles and cutaneous lesions in rheumatoid vasculitis patients and a correlation between the inhibitor KIR2DL3 and the HLA-C*0802 ligand in rheumatoid vasculitis patients were found. CONCLUSION: An association was found between the presence of the HLA-DRB1*1402 and HLA-DRB1*0101 alleles and the development of cutaneous lesions in rheumatoid vasculitis patients. Additionally, the HLA-C*0802 ligand protects these individuals from developing cutaneous lesions. .

The case of a boy with nevus of Ota, extensive Mongolian spot, nevus flammeus, nevus anemicus and cutis marmorata telangiectatica congenita: a unique instance of phacomatosis pigmentovascularis

Phacomatosis pigmentovascularis is a rare, congenital condition characterized by a combination of cutaneous melanocytic lesions and vascular malformation. We discuss an entirely unique case of Phacomatosis pigmentovascularis with nevus of Ota, extensive Mongolian spot, nevus flammeus, nevus anemicus and cutis marmorata telangiectatica congenita, which may represent a heretofore undescribed variant of phacomatosis pigmentovascularis.

Angioma serpiginosum: report of an unusual acral case and review of the literature

We report the case of a 35-year-old woman with deep-red asymptomatic macules on the plantar and dorsal skin of the right great toe. Histopathologic fi ndings were compatible with Angioma serpiginosum. Immunohistochemical stains for estrogens and progesterone receptors were negative. Dermoscopy showed an erythematous parallel ridge pattern with double rows of irregular dots and globules. We report an unusual case of angioma serpiginosum with acral volar skin involvement. The dermoscopic features described may aid in the diagnosis of AS in this specifi c skin area. Acral volar skin involvement must be included in the clinical spectrum of Angioma serpiginosum and in the differential diagnosis of acral vascular lesions.