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1.
Internist (Berl) ; 60(8): 805-813, 2019 Aug.
Artigo em Alemão | MEDLINE | ID: mdl-31300830

RESUMO

Cutaneous vasculitides present with typical clinical signs depending on the size and distribution of the affected vessels. Since there are no large vessels in the skin, giant cell arteritis and Takayasu's arteritis only rarely lead to cutaneous symptoms. The classical systemic polyarteritis nodosa (PAN) is very rare. More frequent is cutaneous PAN presenting with a typical localized livedo racemosa and palpable subcutaneous nodules. The ANCA-associated vasculitides, which belong to the small vessel vasculitides, usually show systemic involvement and manifest on the skin with a diverse picture. Immune complex vasculitides are characterized by deposition of immunoglobulins on the walls of small vessels. The most common form is IgA vasculitis with the cardinal symptoms of palpable, round or oval and partially branched (retiform) purpura and a clear predilection for the legs. Serum disease is a príme example of systemic immune complex diseases due to large circulating immune complexes. Cryoglobulinemic vasculitis primarily involves small as well as medium sized vessels. Simultaneous involvement of vessels of different sizes is characteristic for vasculitis in systemic lupus erythematosus (SLE), Sjögren's syndrome, rheumatoid arthritis and sarcoidosis. Recurrent macular vasculitis in hypergammaglobulinemia is an episodic macular vasculitis of the small blood vessels. Bacteremic vasculitis of the small vessels (without direct microbial infection of the vessel wall) can typically also affect capillaries of dermal papillae and is usually accompanied by a disseminated intravascular coagulation (DIC). In some cases more than one distinct cutaneous vasculitis can occur in the same patient.


Assuntos
Artrite Reumatoide/patologia , Arterite de Células Gigantes/patologia , Livedo Reticular/patologia , Dermatopatias Vasculares/diagnóstico , Dermatopatias/diagnóstico , Dermatopatias/etiologia , Pele/patologia , Eritema/patologia , Humanos , Púrpura/patologia , Púrpura de Schoenlein-Henoch
2.
Internist (Berl) ; 60(8): 799-804, 2019 Aug.
Artigo em Alemão | MEDLINE | ID: mdl-31278434

RESUMO

The skin is one of the organs most commonly affected by vasculitis and the only one that is open to direct inspection. Cutaneous vasculitis can be part of a systemic vasculitis, a variant restricted to the skin (e.g. systemic and cutaneous IgA1 vasculitis) or an independent cutaneous form (recurrent macular vasculitis in hypergammaglobulinemia, nodular vasculitis). For the nomenclature and classification of cutaneous vasculitides the scheme of the Chapel Hill Consensus Conference can be used and the vessels mainly affected determine the clinical picture of individual forms of vasculitis. Some cutaneous efflorescences and their distribution are so characteristic for certain forms of vasculitis that they provide diagnostic indications or even diagnostic criteria. A palpable purpura on the legs is typical for involvement of postcapillary venules in the context of immune complex vasculitis. If arterioles or venules in the dermis are additionally affected, the clinical presentation is that of plaques with marginal offshoots and central hemorrhagic blisters or necrosis (retiform purpura). In contrast to the purpura in occluding vasculopathies, which shows no or little surrounding erythema, the purpura in vasculitis is accompanied by an inflammatory erythema. In vasculitides of the arterioles and small-caliber arteries in the subcutaneous tissue, visible or only palpable nodules exist surrounded by an irregular livedo or also retiform purpura. Understanding how vasculitis-induced efflorescence arises and the histological manifestations helps their recognition during physical examinations in daily practice and is an important guide in the diagnosis and classification of vasculitides.


Assuntos
Exame Físico/métodos , Dermatopatias Vasculares/diagnóstico , Dermatopatias/diagnóstico , Dermatopatias/etiologia , Pele/patologia , Vasculite/classificação , Vasculite/diagnóstico , Eritema , Humanos , Púrpura , Púrpura de Schoenlein-Henoch , Terminologia como Assunto , Vasculite/complicações , Vasculite/imunologia
3.
Cutis ; 103(6): 336-339, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31348448

RESUMO

Acroangiodermatitis (AAD), also known as pseudo-Kaposi sarcoma, is a rare benign vascular proliferation mainly of the extremities. It is characterized by violaceous patches or plaques resembling Kaposi sarcoma. The term pseudo-Kaposi sarcoma encompasses 2 variants of acroangiodermatitis: Mali type and Stewart-Bluefarb syndrome (SBS). Mali-type AAD is more common and is associated with chronic venous hypertension, while SBS is more rare and is associated with arteriovenous malformations and iatrogenic arteriovenous fistulae. We report 2 patients, representing each type of AAD.


Assuntos
Acrodermatite/diagnóstico , Fístula Arteriovenosa/diagnóstico , Malformações Arteriovenosas/diagnóstico , Dermatopatias Vasculares/diagnóstico , Acrodermatite/patologia , Idoso , Fístula Arteriovenosa/patologia , Malformações Arteriovenosas/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Sarcoma de Kaposi/diagnóstico , Sarcoma de Kaposi/patologia , Dermatopatias Vasculares/patologia , Síndrome
4.
J Spec Oper Med ; 19(2): 14-17, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31201746

RESUMO

The initial livedo skin changes of cutis marmorata, also known as cutaneous decompression sickness (DCS), are transient in nature. Accordingly, early images of violaceous skin changes with variegated, marbled, or mottled appearance are rare, whereas later images of deep, erythematous, or violaceous skin changes are readily available. This case presents the opportunity to view the early skin changes characteristic of cutaneous DCS, which would likely manifest at Level I care in the setting of a diving injury during Special Operations missions in austere environments. The unique diving context also allows an overview of DCS in addition to a review of skin eruptions associated with various marine life. As diving is frequently used by Naval Special Warfare, topics presented in this case have significant relevance to Special Operations.


Assuntos
Doença da Descompressão/diagnóstico , Mergulho/efeitos adversos , Dermatopatias Vasculares/diagnóstico , Exantema , Humanos , Militares
5.
Cutis ; 103(3): 181-184, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31039226

RESUMO

Diffuse dermal angiomatosis (DDA) is a benign and rare acquired, cutaneous, reactive, vascular disorder. We report a rare case of a 43-year-old man who presented with a large (15-cm diameter), indurated, hyperpigmented plaque covering the left buttock for 6 years. This report further discusses DDA with a review of the literature, including its classification, epidemiology, pathophysiology, etiology, histopathology, differential diagnosis, and current therapeutic approaches.


Assuntos
Angiomatose/diagnóstico , Dermatopatias Vasculares/diagnóstico , Adulto , Angiomatose/etiologia , Angiomatose/fisiopatologia , Angiomatose/terapia , Humanos , Masculino , Dermatopatias Vasculares/etiologia , Dermatopatias Vasculares/fisiopatologia , Dermatopatias Vasculares/terapia
7.
Rev Med Suisse ; 15(645): 713-718, 2019 Apr 03.
Artigo em Francês | MEDLINE | ID: mdl-30942968

RESUMO

Purpura can be a clinical manifestation of various diseases. The causes of purpura are divided into two main categories: thrombocytopenia and vasculopathies. Cutaneous vasculitis belongs to the latter group. Cutaneous vasculitis should be considered a symptom rather than a medical entity. Some forms of cutaneous vasculitis are limited to the skin and are known as isolated cutaneous vasculitis, while other forms may be part of a systemic disease with a more serious prognosis. It is essential to clarify the type and severity of the disease for optimal patient care. A delay in the identification and start of treatment can be the cause of serious and potentially irreversible complications. Through this article, we will propose a step-by-step approach from diagnosis to patient care.


Assuntos
Púrpura , Dermatopatias Vasculares , Vasculite , Adulto , Humanos , Prognóstico , Púrpura/diagnóstico , Dermatopatias Vasculares/diagnóstico , Vasculite/diagnóstico
8.
Ital J Pediatr ; 45(1): 53, 2019 Apr 27.
Artigo em Inglês | MEDLINE | ID: mdl-31029177

RESUMO

BACKGROUND: Angioma serpiginosum is a rare vascular anomaly whose pathogenesis is still unknown. It is characterized by the onset of vascular reddish macules and papules during childhood, lesions are usually monolateral with a linear serpiginous pattern. It is rarely associated with extracutaneous findings. This entity has not yet been included in the classification of the International Society for the Study of Vascular Anomalies. CASE PRESENTATION: We describe the first Italian report of angioma serpiginosum with a congenital symmetrical presentation. The patient had a further extension of macules during puberty involving both of the soles. No extracutaneous manifestations were present. Diagnosis was confirmed with dermoscopy and light microscopy that revealed the typical clusters of dilated, thickened and PAS+ capillaries in the upper dermis. Moreover, Immunohistochemistry showed positive WT-1 staining. Genetic analysis with next generation sequencing did not detected any mutation. CONCLUSIONS: Our patient presented a peculiar symmetrical and planar extension with a serpiginous linear pattern. The proliferative nature of this condition has been widely discussed in literature. In our case immunohistochemistry was positive for Wilms tumor-1, a new endothelial marker expressed during angiogenesis in reparative processes and endothelial tumors. Clinical evolution, histological and immunohistochemical findings suggest that angioma serpiginosum should be considered as a vascular proliferation. For these reasons we think it should be included in the international classification as a tumor.


Assuntos
Doenças Genéticas Ligadas ao Cromossomo X/diagnóstico , Dermatopatias Vasculares/congênito , Adolescente , Criança , Doenças Genéticas Ligadas ao Cromossomo X/terapia , Humanos , Masculino , Dermatopatias Vasculares/diagnóstico , Dermatopatias Vasculares/terapia
9.
Dermatol Online J ; 25(2)2019 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-30865408

RESUMO

Calcific uremic arteriolopathy (CUA) or calciphylaxis is a syndrome characterized by calcification of vessels located in the dermis and adipose tissue. It commonly occurs in patients with diabetes mellitus, hypertension, and end-stage renal disease. Clinical presentation generally begins with severe pain, followed by the presence of liveloid or purpuric plaques. Later the formation necrotic ulcers occur. This condition is associated with a poor prognosis, with a high rate of mortality within months of the diagnosis. Penile involvement is an uncommon but severe manifestation. We present an 81-year-old man with a history of diabetes mellitus, hypertension, and end-stage renal disease with a one-month evolution of painful necrotic ulcers on his glans penis. He was diagnosed with CUA. Owing to infection complicated by sepsis; penectomy was performed. Unfortunately, the patient died of myocardial infarction during his hospitalization.


Assuntos
Calciofilaxia/complicações , Calciofilaxia/diagnóstico , Doenças do Pênis/etiologia , Dermatopatias Vasculares/complicações , Úlcera Cutânea/etiologia , Pele/patologia , Idoso de 80 Anos ou mais , Humanos , Falência Renal Crônica/complicações , Masculino , Necrose/etiologia , Doenças do Pênis/patologia , Dermatopatias Vasculares/diagnóstico , Uremia/complicações
12.
Scand J Rheumatol ; 48(4): 308-314, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30672367

RESUMO

Objective: Within the spectrum of polyarteritis nodosa (PAN), cutaneous PAN (cPAN) is further classified into mild cPAN and severe cPAN which presents with ulcers, necrosis, or neuritis. As distinguishing between severe cPAN and systemic PAN can be difficult, this study evaluated the clinical characteristics of patients with necrotizing arteritis of medium-sized arteries. Methods: Forty-one patients diagnosed with necrotizing arteritis of medium-sized arteries between 2008 and 2017 at our institution were enrolled in this study. Clinical background, laboratory findings, treatments, and rates of relapse and death were evaluated. Results: Thirty-six patients were classified as having cPAN (mild, 15; ulcer, nine; neuritis, eight; both, four), and five cases manifested systemic vasculitis. Clinical characteristics of mild cPAN included female predominance (84.6%) and younger age (median 31 years); those of systemic PAN included older age (median 71 years) and higher levels of inflammatory markers. Severe cPAN manifested with intermediate phenotypes. The median doses of prednisolone used to treat mild cPAN, severe cPAN, and systemic PAN were 20.0, 40.0, and 40.0 mg/day, respectively. Immunosuppressants were used in 20.0% of mild cPAN, 90.5% of severe cPAN, and 80.0% of systemic PAN patients. Although the mortality rates were indistinguishable, the relapse rates of severe cPAN (ulcer type) were significantly higher than those of other types (88.9%). Conclusion: The clinical characteristics of mild cPAN, severe cPAN (ulcer type), severe cPAN (neuritis type), and systemic PAN were distinct from each other. In particular, patients with severe cPAN (ulcer type) had higher relapse rates, indicating the importance of combination therapy.


Assuntos
Artérias , Imunossupressores/uso terapêutico , Inflamação/diagnóstico , Poliarterite Nodosa , Dermatopatias Vasculares/diagnóstico , Vasculite Sistêmica/diagnóstico , Adulto , Fatores Etários , Idoso , Artérias/imunologia , Artérias/patologia , Correlação de Dados , Feminino , Humanos , Japão/epidemiologia , Masculino , Fenótipo , Poliarterite Nodosa/diagnóstico , Poliarterite Nodosa/imunologia , Poliarterite Nodosa/mortalidade , Poliarterite Nodosa/fisiopatologia , Recidiva , Índice de Gravidade de Doença , Dermatopatias Vasculares/tratamento farmacológico , Vasculite Sistêmica/tratamento farmacológico
13.
Medicine (Baltimore) ; 98(3): e14121, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30653140

RESUMO

RATIONALE: Cutaneous vasculitis is usually found in patients with rheumatoid arthritis (RA) as an extra-articular manifestation, but rare in patients with ankylosing spondylitis (AS). In this case, we describe an AS patient who developed large skin lesions, of which the histological findings were consistent with cutaneous vasculitis. PATIENT CONCERNS: A 22-year-old male who was diagnosed as HLA-27 positive AS for 5 years. However, in the last year, he suffered a recurrent skin lesion in both lower extremities especially the dorsum of feet, accompanying with intense pruritus. DIAGNOSIS: The originally diagnosis of the skin lessons was dermatitis, and then a skin biopsy pathology showed it was consistent with cutaneous vasculitis. INTERVENTION: At first he was treated with gentamycin and ethacridine for nearly 2 weeks, the symptoms were relieved. But then he suffered severe cutaneous lesions with swell at the ulcerous margin in his dorsum of right foot. The patient was treated with dexamethasone 10 mg intravenous drip daily for 2 weeks, and followed by methylprednisolone 160 mg oral administration daily after discharge. The skin lesions were gradually healed in several months OUTCOMES:: This patient was followed up at 1-month, 5-month, and 10-month after discharged, the skin lessons was gradually healed and never occurred. LESSONS: This case indicated that cutaneous vasculitis might be an extra-articular manifestation of AS in which IgA may plays a pathogenic part. All this may be associated with the damage of cytokines and autoantibodies to vascular endothelial wall caused by active inflammatory phase.


Assuntos
Dermatopatias Vasculares/etiologia , Espondilite Anquilosante/complicações , Vasculite/etiologia , Dermatite/diagnóstico , Diagnóstico Diferencial , Pé/patologia , Humanos , Masculino , Pele/patologia , Dermatopatias Vasculares/diagnóstico , Vasculite/diagnóstico , Adulto Jovem
15.
Curr Opin Rheumatol ; 31(1): 46-52, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30394939

RESUMO

PURPOSE OF REVIEW: Cutaneous vasculitis reflects a spectrum ranging from skin limited to severe systemic forms. To date, there is still no generally acknowledged nomenclature for cutaneous vasculitis. This review aims to summarize the recent advances in the nomenclature of cutaneous vasculitis. RECENT FINDINGS: The most widely adopted vasculitis classification system is the one of 2012 Revised Chapel Hill Consensus Conference (CHCC) which represent not such a classification but a nomenclature system that name vasculitis on the basis of the size of the vessel affected. The CHCC 2012 did not deal with the special features of cutaneous vasculitis and did not explicitly discuss the presence of skin-limited or skin-dominant forms of vasculitis. Therefore, a consensus group was formed to propose an Addendum to CHCC 2012, focusing on cutaneous vasculitis. The Addendum better clarify the main aspects of some single-organ vasculitis, including IgM/IgG vasculitis, nodular vasculitis, erythema elevatum et diutinum and recurrent macular vasculitis in hypergammaglobulinemia. Moreover, it differentiated normocomplementemic from hypocomplementemic urticarial vasculitis. Finally, it recognized cutaneous polyarteritis nodosa as a distinct subtype of polyarteritis nodosa. SUMMARY: Classification criteria are useful tools to standardize names and definitions for cutaneous vasculitis; however, they do not represent diagnostic criteria. Collaborative efforts are still needed to get a shared classification and valid diagnostic criteria for cutaneous vasculitis.


Assuntos
Dermatopatias Vasculares/diagnóstico , Pele/patologia , Vasculite/diagnóstico , Humanos , Dermatopatias Vasculares/classificação , Dermatopatias Vasculares/patologia , Terminologia como Assunto , Vasculite/classificação , Vasculite/patologia
17.
J Cutan Pathol ; 46(2): 143-147, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30362142

RESUMO

Cutaneous vasculitis has many underlying causes, and the clinical and histological findings often overlap. Inflammatory vasculitis can mimic infection; however, distinction is critical for the timely institution of appropriate therapy. We present two patients who had generalized polymorphous eruptions whose cutaneous pathology showed vasculitis with unusual haloed yeast-like cells within the inflammatory infiltrate, mimicking Cryptococcus. The unusual cells stained negatively with Gomori methenamine silver and periodic acid-Schiff fungal stains, but positively for CD68 and had cytoplasmic reactivity with antibody to myeloperoxidase (MPO). Both patients had positive serum anti-MPO antibodies. The first patient experienced a rapidly fatal course, whereas the second patient improved with prompt initiation of systemic corticosteroids. Interestingly, the second case had prior biopsy showing Sweet syndrome with crypotoccoid-appearing cells. Cryptococcoid cells have been described previously in association with neutrophilic dermatoses, but not in the setting of vasculitis as was seen in our patients. Our cases add to the existing literature on crypotoccoid mimickers, and are the first to be reported in association with vasculitis.


Assuntos
Criptococose , Cryptococcus , Dermatomicoses , Dermatopatias Vasculares , Síndrome de Sweet , Vasculite , Idoso , Criptococose/diagnóstico , Criptococose/metabolismo , Criptococose/patologia , Dermatomicoses/diagnóstico , Dermatomicoses/metabolismo , Dermatomicoses/patologia , Feminino , Humanos , Dermatopatias Vasculares/diagnóstico , Dermatopatias Vasculares/metabolismo , Dermatopatias Vasculares/patologia , Síndrome de Sweet/diagnóstico , Síndrome de Sweet/metabolismo , Síndrome de Sweet/patologia , Vasculite/diagnóstico , Vasculite/metabolismo , Vasculite/patologia
18.
Fetal Diagn Ther ; 45(4): 281-284, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-29791897

RESUMO

We report a case of a fetus diagnosed at 28 weeks' gestation with a spontaneous prenatal hemothorax. Fetal intervention consisted of 2 thoracenteses with analysis of the pleural effusion. The pregnancy was further complicated by recurrence of the hemothorax, with subsequent mediastinal shift, hydrops, and nonreassuring antenatal testing requiring delivery at 31 weeks' gestation. Postnatal workup established the diagnoses of cutis marmorata telangiectatica congenita (CMTC) and pulmonary lymphangiectasia. The child is currently 4 years old and without any active medical issues or sequelae from the CMTC, pulmonary lymphangiectasia, or prenatal interventions.


Assuntos
Hemotórax/diagnóstico por imagem , Dermatopatias Vasculares/diagnóstico , Telangiectasia/congênito , Diagnóstico Diferencial , Feminino , Humanos , Gravidez , Diagnóstico Pré-Natal , Dermatopatias Vasculares/complicações , Telangiectasia/complicações , Telangiectasia/diagnóstico
19.
Int J Dermatol ; 58(7): 782-787, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-30238440

RESUMO

Gardner-Diamond syndrome (GDS) is a rare psychodermatological condition characterized by the formation of spontaneous, painful skin lesions that develop into ecchymosis following episodes of severe physiological or psychological stress. The majority of GDS cases occur in young adult females, and although the etiology of this rare disorder is unknown, there appears to be a psychological component correlated with the coexistence of previous psychiatric diagnoses. Due to the rare nature of this disorder, there exist few guidelines for prompt clinical diagnosis and optimal treatment. Here, a systematic review was conducted to include 45 cases of patients with GDS to better understand clinical presentation as well as current treatment options. Ultimately, GDS is a diagnosis of exclusion after other coagulopathies and causes of purpura are ruled out. High clinical suspicion following laboratory and clinical exclusion of known physiological causes is necessary for diagnosis. Selective serotonin reuptake inhibitors (SSRIs) and corticosteroids are cost effective first line treatments for GDS with proven efficacy in symptomatic relief. GDS refractory to initial treatment may require regular psychotherapy and titrated SSRI dosages to achieve long-term success. This review of available case studies serves to comprehensively describe the clinical presentation and available treatment approaches to this rare psychodermatological disorder.


Assuntos
Doenças Autoimunes/terapia , Transtornos Autoinduzidos/terapia , Glucocorticoides/administração & dosagem , Psicoterapia , Transtornos Psicóticos/terapia , Doenças Raras/terapia , Inibidores de Captação de Serotonina/administração & dosagem , Dermatopatias Vasculares/terapia , Adolescente , Adulto , Idade de Início , Doenças Autoimunes/diagnóstico , Criança , Relação Dose-Resposta a Droga , Transtornos Autoinduzidos/diagnóstico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Transtornos Psicóticos/diagnóstico , Doenças Raras/diagnóstico , Fatores Sexuais , Dermatopatias Vasculares/diagnóstico , Adulto Jovem
20.
Dermatol Clin ; 37(1): 37-48, 2019 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-30466687

RESUMO

Connective tissue diseases often prominently affect the skin, requiring dermatologists to play an important role in diagnosis and treatment of these patients. Herein we describe updates on the pathogenesis, clinical features, and treatment of 4 major connective tissue diseases: dermatomyositis, cutaneous lupus erythematosus, limited scleroderma (morphea), and cutaneous vasculitis. Many of these updates promise to improve clinical care of patients who suffer from dermatologic involvement of these diseases and are the result of research performed by dermatologists who have expertise in these conditions.


Assuntos
Dermatomiosite/classificação , Dermatomiosite/tratamento farmacológico , Lúpus Eritematoso Cutâneo/tratamento farmacológico , Esclerodermia Localizada/terapia , Antimaláricos/uso terapêutico , Biomarcadores/sangue , Humanos , Lúpus Eritematoso Cutâneo/fisiopatologia , Púrpura de Schoenlein-Henoch/diagnóstico , Esclerodermia Localizada/sangue , Esclerodermia Localizada/classificação , Dermatopatias Vasculares/classificação , Dermatopatias Vasculares/diagnóstico , Dermatopatias Vasculares/tratamento farmacológico , Vasculite/classificação , Vasculite/diagnóstico , Vasculite/tratamento farmacológico
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