RESUMO
The mechanisms underlying the onset and progression of vasculitis remain poorly understood. This condition is characterized by damage to the vascular wall, recruitment of inflammatory cells, and subsequent structural remodeling, which are hallmarks of vasculitis. The histopathological classification of vasculitis relies on the size of the affected vessel and the predominant type of inflammatory cell involved - neutrophils in acute cases, lymphocytes in chronic conditions, and histiocytes in granulomatous forms. Pathological changes progress in every context, and a single vasculitic pattern can be associated with various systemic conditions. Conversely, a single causative agent may lead to multiple distinct clinical and pathological manifestations of vasculitis. Moreover, many cases of vasculitis have no identifiable cause. A foundational understanding of the normal structure of the cutaneous vascular network is crucial. Similarly, identifying the cellular and molecular participants and their roles in forming the "dermal microvascular unit" is propedeutical.This review aims to elucidate the complex mechanisms involved in the initiation and progression of vasculitis, offering a comprehensive overview of its histopathological classification, underlying causes, and the significant role of the cutaneous vascular network and cellular dynamics. By integrating the latest insights from studies on NETosis and the implications of lymphocytic infiltration in autoimmune diseases, we seek to bridge gaps in current knowledge and highlight areas for future research. Our discussion extends to the clinical implications of vasculitis, emphasizing the importance of identifying etiological agents and understanding the diverse histopathological manifestations to improve diagnostic accuracy and treatment outcomes.
Assuntos
Pele , Vasculite , Humanos , Vasculite/patologia , Vasculite/etiologia , Pele/patologia , Pele/irrigação sanguínea , Neutrófilos/patologia , Linfócitos/patologia , Linfócitos/imunologia , Dermatopatias Vasculares/patologia , Dermatopatias Vasculares/imunologia , Dermatopatias Vasculares/etiologia , Dermatopatias Vasculares/diagnósticoRESUMO
OBJECTIVE: Single-organ cutaneous small-vessel vasculitis (SoCSVV) is an inflammatory skin-limited vascular disease affecting the dermal and/or hypodermal vessel wall. Pathogenetically, idiopathic forms are described, as well as the induction from different triggers, such as infections, drugs, and vaccines. Following the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic outbreak, cases of cutaneous vasculitis induced by both COVID-19 and COVID-19 vaccinations have been reported in literature. The aim of this study is to provide the most recent evidence on new etiological factors, clinical features, and management of the SoCSVV. PATIENTS AND METHODS: We included 42 patients (22 women, 20 men) with SoCSVV and no systemic involvement in the study. The mean age of the patients was 57.3 years. Palpable purpura was the most frequent clinical manifestation (38 cases-90.4%). All patients were diagnosed with leukocytoclastic vasculitis by skin biopsy. RESULTS: The etiological factors were as follows: idiopathic in 9 (21%) patients, drug-related in 19 (45%) patients, COVID-19 infection-related in 5 (12%) patients, post-COVID-19 vaccination in 5 (12%) patients, paraneoplastic in 2 (5%) patients, and drug and infection and sepsis in 1 patient each. Among the drug-related cases, 16 (84%) were antibiotic-related, and most of them were beta-lactam antibiotics. Eosinophilia was present in skin biopsy in the cases related to vaccination and drugs, while intense necrosis and vascular damage in the skin were observed in the cases related to COVID-19 infection, unlike the others. A rapid resolution was observed with the cessation of drugs and short-term steroid treatment for the precipitating factors. CONCLUSIONS: SoCSVV is usually associated with drugs, preceding infections, and vaccines. COVID-19 infection and COVID-19 vaccinations have been reported as new etiological factors. SoCSVV indicates that the disease seems to be a mild, self-limiting illness with a good clinical result.
Assuntos
COVID-19 , Dermatopatias Vasculares , Vasculite Leucocitoclástica Cutânea , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , COVID-19/complicações , Vacinas contra COVID-19/efeitos adversos , Pele/patologia , Dermatopatias Vasculares/diagnóstico , Dermatopatias Vasculares/etiologia , Dermatopatias Vasculares/patologia , Vasculite Leucocitoclástica Cutânea/diagnóstico , Vasculite Leucocitoclástica Cutânea/etiologia , Vasculite Leucocitoclástica Cutânea/patologiaRESUMO
OBJECTIVES: To describe the clinical and pathological features of biopsy-proven cutaneous vasculitis (CV) associated with SLE, focusing on diagnosis classification and impact on overall SLE activity. METHODS: Retrospective multicentric cohort study including SLE patients with biopsy-proven CV identified by (i) data from pathology departments of three university hospitals and (ii) a national call for cases. SLE was defined according to 1997 revised ACR and/or 2019 ACR/EULAR criteria. CV diagnosis was confirmed histologically and classified by using the dermatological addendum of the Chapel Hill classification. SLE activity and flare severity at the time of CV diagnosis were assessed independently of vasculitis items with the SELENA-SLEDAI and SELENA-SLEDAI Flare Index. RESULTS: Overall, 39 patients were included; 35 (90%) were female. Cutaneous manifestations included mostly palpable purpura (n = 21; 54%) and urticarial lesions (n = 18; 46%); lower limbs were the most common location (n = 33; 85%). Eleven (28%) patients exhibited extracutaneous vasculitis. A higher prevalence of Sjögren's syndrome (51%) was found compared with SLE patients without CV from the French referral centre group (12%, P < 0.0001) and the Swiss SLE Cohort (11%, P < 0.0001). CV was mostly classified as urticarial vasculitis (n = 14, 36%) and cryoglobulinaemia (n = 13, 33%). Only 2 (5%) patients had no other cause than SLE to explain the CV. Sixty-one percent of patients had inactive SLE. CONCLUSION: SLE-related vasculitis seems very rare and other causes of vasculitis should be ruled out before considering this diagnosis. Moreover, in more than half of patients, CV was not associated with another sign of active SLE.
Assuntos
Lúpus Eritematoso Sistêmico , Dermatopatias Vasculares , Urticária , Vasculite , Humanos , Feminino , Masculino , Estudos Retrospectivos , Estudos de Coortes , Lúpus Eritematoso Sistêmico/diagnóstico , Dermatopatias Vasculares/etiologia , Vasculite/complicações , Urticária/complicaçõesRESUMO
Cutaneous Vasculitides - Clinical Manifestations, Diagnosis, and Aetiology Abstract. Vasculitides are a heterogeneous group of diseases that are classified differently, for example according to the size of the affected vessel or according to primary and secondary causes. The skin is most frequently affected; it can be involved both as single organ vasculitis and in the context of systemic forms. The combination of skin lesions, their anatomical location and information on the time course provide clues for a differential diagnosis. Purpura, blisters, necrosis, ulcerations and possibly a livedo are characteristic manifestations. Constitutional symptoms such as weight loss, exhaustion, fever, and arthralgias are indicative of a systemic form. It is important to differentiate vasculitides from vasculopathies, which can manifest similarly. The most common form in adults is cutaneous leukocytoclastic angiitis, in children IgA vasculitis (Schönlein-Henoch purpura). Various triggers are possible: infections, drugs, autoimmune diseases, and malignancies, whereby up to 50% remain etiologically unexplained. Skin biopsies and laboratory parameters, if necessary supplemented with imaging, are important steps in the clarification process. Treatment is primarily directed at the elimination of a possible triggering cause. Idiopathic cutaneous leukocytoclastic angiitis usually resolves spontaneously; treatment is symptomatic. In more severe cases, topical corticosteroids or calcineurin antagonists are primarily used. In case of therapeutic resistance, systemic immunosuppressants are recommended.
Assuntos
Vasculite por IgA , Dermatopatias Vasculares , Vasculite Leucocitoclástica Cutânea , Vasculite , Adulto , Criança , Diagnóstico Diferencial , Humanos , Vasculite por IgA/diagnóstico , Vasculite por IgA/terapia , Pele/patologia , Dermatopatias Vasculares/diagnóstico , Dermatopatias Vasculares/etiologia , Dermatopatias Vasculares/terapia , Vasculite/diagnóstico , Vasculite/etiologia , Vasculite/terapia , Vasculite Leucocitoclástica Cutânea/diagnóstico , Vasculite Leucocitoclástica Cutânea/etiologia , Vasculite Leucocitoclástica Cutânea/terapiaRESUMO
Retiform purpura has been described as a relatively frequent cutaneous finding in patients with coronavirus disease 2019 (COVID-19). The etiology is hypothesized to be related to thrombotic vasculopathy based on lesional biopsy specimen findings, but the pathogenesis of the vasculopathy is not completely understood. Here, we present a case of a retiform purpuric patch on the sacrum/buttocks in a hospitalized patient prior to subsequent diagnosis of COVID-19 and an eventual fatal disease course. Two lesional biopsy specimens at different time points in the disease course revealed thrombotic vasculopathy, despite therapeutic anticoagulation. Detailed histopathologic evaluation using immunohistochemical markers suggest the etiology of the vasculopathy involves both persistent complement activation and platelet aggregation, which possibly promote ongoing thrombus formation. This case highlights that sacral/buttock retiform purpuric patches may be a presenting sign of infection with SARS-CoV-2 virus and may represent an ominous sign supporting a future severe disease course. In addition, biopsy specimen findings at separate time points demonstrate that cutaneous vasculopathy may persist despite adequate systemic anticoagulation, possibly due to the combination of persistent complement and platelet activation. Finally, occlusive thrombi in sacral/buttock retiform purpuric patches may contribute to future ulceration and significant cutaneous morbidity in patients who survive COVID-19.
Assuntos
Nádegas/patologia , COVID-19/complicações , COVID-19/patologia , Púrpura/diagnóstico , Sacro/patologia , Idoso , Anticoagulantes/uso terapêutico , Biópsia/métodos , Nádegas/virologia , COVID-19/diagnóstico , COVID-19/imunologia , Calciofilaxia/diagnóstico , Ativação do Complemento/imunologia , Diagnóstico Diferencial , Progressão da Doença , Evolução Fatal , Feminino , Humanos , Pacientes Internados , Agregação Plaquetária/imunologia , Púrpura/virologia , SARS-CoV-2/genética , SARS-CoV-2/isolamento & purificação , Sacro/virologia , Pele/patologia , Dermatopatias Vasculares/etiologia , Dermatopatias Vasculares/patologiaAssuntos
Preenchedores Dérmicos/efeitos adversos , Dermatoses Faciais/terapia , Síndrome de Lipodistrofia Associada ao HIV/terapia , Dermatopatias Vasculares/terapia , Administração Cutânea , Terapia Combinada/métodos , Preenchedores Dérmicos/administração & dosagem , Durapatita/administração & dosagem , Durapatita/efeitos adversos , Face/irrigação sanguínea , Dermatoses Faciais/etiologia , Síndrome de Lipodistrofia Associada ao HIV/complicações , Hemofilia A/complicações , Humanos , Masculino , Pessoa de Meia-Idade , Nitroglicerina/administração & dosagem , Fototerapia/métodos , Dermatopatias Vasculares/etiologia , Resultado do TratamentoRESUMO
Vascular occlusion has multiple, diverse clinical manifestations, some of which can have grave consequences for patients. It also has a wide variety of causes, including thrombi, which we recently addressed in partI of this review. In this second part, we look at additional causes of vascular occlusion.
Assuntos
Transtornos da Coagulação Sanguínea/complicações , Embolia/complicações , Dermatopatias Vasculares/etiologia , Anticoagulantes/efeitos adversos , Síndrome Antifosfolipídica/complicações , Síndrome Antifosfolipídica/patologia , Calciofilaxia/complicações , Calciofilaxia/patologia , Cocaína/efeitos adversos , Feminino , Corpos Estranhos/complicações , Corpos Estranhos/patologia , Humanos , Isquemia/etiologia , Isquemia/patologia , Levamisol/efeitos adversos , Livedo Reticular/etiologia , Livedo Reticular/patologia , Masculino , Papulose Atrófica Maligna/patologia , Necrose , Neoplasias/complicações , Neoplasias/patologia , Paraproteinemias/complicações , Paraproteinemias/patologia , Pele/irrigação sanguínea , Dermatopatias Vasculares/induzido quimicamente , Dermatopatias Vasculares/patologia , Úlcera Cutânea/etiologia , Úlcera Cutânea/patologia , Síndrome de Sneddon/patologiaRESUMO
This review examines the clinical, morphological, and systemic factors related to coronavirus disease 2019 (COVID-19) cutaneous manifestations. The EMBASE, Medline, and Pubmed Central databases were searched from February 1, 2020 until April 25, 2020, using the search words "(COVID-19 OR SARS-CoV-2 OR coronavirus-19) AND (skin OR cutaneous OR dermatologic)". Cutaneous manifestations of COVID-19 were included. The cutaneous manifestations can be classified into 2 types. Patients with inflammatory reactions consisted of morbilliform, varicella-like, urticarial eruptions, and vesiculobullous manifestations. These manifestations were mainly found on the trunk, limbs, and faces of patients and had mainly positive COVID-19 polymerase chain reaction findings (97.7%). Furthermore, there were 516 patients with acral vascular lesions: chilblains, livedo lesions, cutaneous small-vessel vasculitis, and other noninflammatory purpura. These were often nonpruritic (88%) and not seen in severe disease (88.7%). The cutaneous lesions have potential for early diagnosis of COVID-19 and prevention of disease transmission. The implications of COVID-19 in the field of dermatology continue to evolve as more clinical data becomes available.
Assuntos
COVID-19/complicações , Diagnóstico Precoce , Pandemias , SARS-CoV-2 , Dermatopatias Vasculares/diagnóstico , COVID-19/epidemiologia , Humanos , Dermatopatias Vasculares/etiologiaRESUMO
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.
Assuntos
Humanos , Feminino , Silicones/efeitos adversos , Histiocitose/etiologia , Histiocitose/patologia , Dermatopatias Vasculares/etiologia , Dermatopatias Vasculares/patologia , Implante Mamário/efeitos adversos , Imuno-Histoquímica , Trombose Venosa/etiologia , Trombose Venosa/patologia , Dilatação Patológica , Pessoa de Meia-IdadeRESUMO
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.
Assuntos
Implante Mamário/efeitos adversos , Histiocitose/etiologia , Histiocitose/patologia , Silicones/efeitos adversos , Dermatopatias Vasculares/etiologia , Dermatopatias Vasculares/patologia , Dilatação Patológica , Feminino , Humanos , Imuno-Histoquímica , Pessoa de Meia-Idade , Trombose Venosa/etiologia , Trombose Venosa/patologiaRESUMO
OBJECTIVE: To investigate the clinicopathologic features of eosinophilic granulomatosis with polyangiitis (EGPA)-associated neuropathy with a focus on the presence or absence of anti-neutrophil cytoplasmic antibodies (ANCAs). METHODS: We examined the clinical features and pathologic findings of sural nerve biopsy specimens from 82 patients with EGPA-associated neuropathy. Of these patients, 32.9% were myeloperoxidase (MPO)-ANCA positive, and 67.1% were MPO-ANCA negative. PR3-ANCA was negative in all of 78 examined patients. RESULTS: Upper limb symptoms were more frequently reported as initial neuropathic manifestations in the MPO-ANCA-positive group than in the MPO-ANCA-negative group (44.4% vs 14.6%, p < 0.01). The serum levels of C-reactive protein were significantly higher in the MPO-ANCA-positive group than in the MPO-ANCA-negative group (p < 0.05). Sural nerve biopsy specimens showed findings suggestive of vasculitis (i.e., destruction of vascular structures) in epineurial vessels; these results were seen more frequently in the MPO-ANCA-positive group than in the MPO-ANCA-negative group (p < 0.0001). Conversely, the numbers of eosinophils in the lumen of the epineurial vessels (p < 0.01) and epineurial vessels occluded by intraluminal eosinophils (p < 0.05) were higher in the MPO-ANCA-negative group than in the MPO-ANCA-positive group. Furthermore, the incidence of eosinophil infiltration in the endoneurium was higher in the MPO-ANCA-negative group than in the MPO-ANCA-positive group (p < 0.01). CONCLUSIONS: This study suggests that the pathogenesis of EGPA comprises at least 2 distinct mechanisms: ANCA-associated vasculitis resulting in ischemic effects and inflammation, which is prominent in MPO-ANCA-positive patients, and eosinophil-associated vascular occlusion leading to ischemia and eosinophil-associated tissue damage, which is conspicuous in MPO-ANCA-negative patients.
Assuntos
Anticorpos Anticitoplasma de Neutrófilos/imunologia , Síndrome de Churg-Strauss/fisiopatologia , Debilidade Muscular/fisiopatologia , Nervos Periféricos/irrigação sanguínea , Doenças do Sistema Nervoso Periférico/fisiopatologia , Distúrbios Somatossensoriais/fisiopatologia , Idoso , Asma/etiologia , Síndrome de Churg-Strauss/complicações , Síndrome de Churg-Strauss/imunologia , Eletrodiagnóstico , Feminino , Humanos , Nefropatias/etiologia , Extremidade Inferior/inervação , Pneumopatias/etiologia , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Debilidade Muscular/etiologia , Mieloblastina/imunologia , Condução Nervosa , Otorrinolaringopatias/genética , Nervos Periféricos/patologia , Doenças do Sistema Nervoso Periférico/etiologia , Doenças do Sistema Nervoso Periférico/imunologia , Doenças do Sistema Nervoso Periférico/patologia , Peroxidase/imunologia , Dermatopatias Vasculares/etiologia , Distúrbios Somatossensoriais/etiologia , Nervo Sural/patologia , Tomografia Computadorizada por Raios X , Extremidade Superior/inervaçãoAssuntos
Neurofibromatose 1/diagnóstico , Dermatopatias Vasculares/diagnóstico , Úlcera Cutânea/etiologia , Administração Cutânea , Adulto , Dorso , Biópsia , Humanos , Masculino , Neurofibromatose 1/complicações , Nitroglicerina/administração & dosagem , Pele/irrigação sanguínea , Pele/patologia , Dermatopatias Vasculares/tratamento farmacológico , Dermatopatias Vasculares/etiologia , Dermatopatias Vasculares/patologia , Úlcera Cutânea/tratamento farmacológico , Úlcera Cutânea/patologiaAssuntos
Angiomatose/etiologia , Doença Enxerto-Hospedeiro/etiologia , Dermatopatias Vasculares/etiologia , Pele/patologia , Antagonistas Adrenérgicos beta/uso terapêutico , Inibidores da Angiogênese/farmacologia , Inibidores da Angiogênese/uso terapêutico , Angiomatose/patologia , Angiomatose/terapia , Doença Enxerto-Hospedeiro/tratamento farmacológico , Doença Enxerto-Hospedeiro/patologia , Humanos , Alvo Mecanístico do Complexo 1 de Rapamicina/antagonistas & inibidores , Sirolimo/farmacologia , Sirolimo/uso terapêutico , Pele/irrigação sanguínea , Dermatopatias Vasculares/patologia , Dermatopatias Vasculares/terapia , Fator A de Crescimento do Endotélio Vascular/antagonistas & inibidoresRESUMO
Retiform purpura is a specific morphology within the spectrum of reticulate eruptions of vascular origin. It develops when blood vessels serving the skin are compromised resulting in downstream cutaneous ischemia, purpura, and necrosis. Identifying retiform purpura is important particularly in the acutely ill patient. It may elucidate the underlying diagnosis, provide prognostic information, and suggest a treatment approach. The differential diagnosis of retiform purpura is vast, reflecting the myriad conditions that can lead to cutaneous vessel wall damage or lumen occlusion. In this article, we give an overview of the differential diagnosis of this cutaneous morphology, provide an approach to workup, and highlight updates in treatment of some of the more common conditions that manifest as retiform purpura.
Assuntos
Púrpura/diagnóstico , Dermatopatias Vasculares/diagnóstico , Biópsia , Técnicas de Laboratório Clínico , Diagnóstico Diferencial , Humanos , Anamnese , Exame Físico , Púrpura/etiologia , Púrpura/patologia , Púrpura/terapia , Dermatopatias Vasculares/etiologia , Dermatopatias Vasculares/patologia , Dermatopatias Vasculares/terapiaRESUMO
Diabetes is a serious, chronic disease with a rising prevalence worldwide. Its complications are a major cause of morbidity and mortality and contribute substantially to health care costs. In this article the authors review the most common and sensitive skin manifestations that can be present on patients with diabetes and prediabetes. The prompt recognition of these frequently underestimated entities is extremely important as it may trigger not only an adequate metabolic evaluation but also a timely referral and appropriate treatment, minimizing the secondary effects of long-term diabetes and improving the prognosis of diabetic patients.
A diabetes mellitus é uma doença crónica, com uma prevalência crescente a nível mundial. As complicações da diabetes são uma causa major de morbilidade e mortalidade, condicionando custos importantes na área da saúde. Neste artigo é efetuada uma revisão das manifestações cutâneas mais frequentes presentes em doentes com diabetes e pré-diabetes. O reconhecimento atempado destas entidades é fulcral, levando não só a uma avaliação atempada do perfil metabólico como a uma referenciação e tratamento precoces. Desta forma, é possível minimizar os efeitos secundários da diabetes a longo prazo, melhorando significativamente o prognóstico dos doentes.
Assuntos
Complicações do Diabetes/etiologia , Estado Pré-Diabético/complicações , Dermatopatias/etiologia , Acantose Nigricans/etiologia , Vesícula/etiologia , Angiopatias Diabéticas/complicações , Pé Diabético/etiologia , Granuloma Anular/etiologia , Humanos , Necrobiose Lipoídica/etiologia , Escleredema do Adulto/etiologia , Dermatopatias Infecciosas/etiologia , Dermatopatias Vasculares/etiologia , Xantomatose/etiologiaRESUMO
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/terapiaRESUMO
Systemic and localized vasculitis affects the skin and subcutis, due to large vascular beds and hemodynamic factors, such as stasis in lower extremities, and environmental influences, as occur in cold exposure. Initial cutaneous manifestations of vasculitides include diverse and dynamic patterns of discoloration, swelling, hemorrhage, and necrosis. One-half of affected patients present with localized, self-limited disease to the skin without any known trigger or associated systemic disease, known as idiopathic cutaneous leukocytoclastic vasculitis. Skin biopsy and dermatopathology contribute relevant information; however, they require correlation with clinical history, physical examination, and laboratory findings to reach an accurate diagnosis.
Assuntos
Dermatopatias Vasculares/etiologia , Dermatopatias Vasculares/patologia , Vasculite Sistêmica/complicações , HumanosRESUMO
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 10âmg intravenous drip daily for 2 weeks, and followed by methylprednisolone 160âmg 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.