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.

Chronic polyarthritis as the first manifestation of childhood systemic polyarteritis nodosa / Poliartrite crônica como primeira manifestação de poliarterite nodosa sistêmica pediátrica

ABSTRACT Arthritis has been reported as an acute pattern, generally evanescent with oligoarthritis, mostly affecting knees and ankles in childhood systemic polyarteritis nodosa. However, chronic polyarthritis with morning stiffness mimicking juvenile idiopathic arthritis has not been reported. We describe the case of a 4-year old girl who had additive and chronic polyarthritis with edema, tenderness, pain on motion and morning stiffness for 2 months. After 45 days, she also presented painful subcutaneous nodules and erythematous-violaceous lesions in the extensor region of upper and lower limbs. She was admitted to university hospital due to high fever, malaise, myalgia, anorexia, loss of weight (1kg), painful skin lesions and severe functional disability. She was bedridden by chronic polyarthritis with limitation on motion. Systolic and diastolic blood pressures were greater than 95th percentile for height. Urine protein/creatinine ratio was 0.39g/day, and immunological tests were negative. Anti-streptolysin O was 1,687UI/mL. Skin biopsy revealed necrotizing vasculitis in medium- and small-sized vessels compatible with polyarteritis nodosa. Therefore, we had the diagnosis of systemic polyarteritis nodosa. Prednisone 2mg/kg/day was administered with complete resolution of skin lesions and arthritis, and improvement of proteinuria (0.26g/day) after 15 days. The diagnosis of childhood systemic polyarteritis nodosa should be considered for patients with chronic polyarthritis associated to cutaneous vasculitis triggered by streptococcal infection.

RESUMO Na poliarterite nodosa sistêmica pediátrica, a artrite caracteriza-se pelo padrão agudo, geralmente evanescente, com oligoartrite, e afeta principalmente joelhos e tornozelos. No entanto, a poliartrite crônica com rigidez matinal e simulando artrite idiopática juvenil ainda não foi relatada. Descrevemos o caso de uma menina de 4 anos que apresentou poliartrite crônica aditiva com edema, dor à palpação e movimento, e rigidez matinal por 2 meses. Após 45 dias, também apresentou nódulos subcutâneos dolorosos e lesões eritêmato-violáceas na região extensora dos membros superiores e inferiores. Foi internada no hospital universitário por conta de febre alta, mal-estar, mialgia, anorexia, perda de peso (1kg), lesões de pele muito dolorosas e incapacidade funcional grave. Estava restrita ao leito devido à poliartrite crônica com limitação do movimento. Pressões sistólica e diastólica foram maiores que percentil 95 para altura. Relação proteína/creatinina urinária estava 0,39g/dia, e os testes imunológicos foram negativos. Antiestreptolisina O era 1.687UI/mL. A biópsia de pele revelou vasculite necrosante de vasos de pequeno e médio calibre, compatível com poliarterite nodosa. Portanto, foi realizado o diagnóstico de poliarterite nodosa sistêmica. Foi administrada prednisona 2mg/kg/dia com resolução completa das lesões de pele e da artrite, além de melhora da proteinúria (0,26g/dia) após 15 dias. O diagnóstico de poliarterite nodosa sistêmica pediátrica deve ser considerado em pacientes com poliartrite crônica associado a lesões cutâneas vasculíticas, sendo a infecção estreptocócica um importante fator desencadeante.

Cutaneous polyarteritis nodosa causing refractory skin deformation and pigmentation as sequel

Abstract: A 39-year-old woman presented with prominent and painful livedo reticularis lesions spreading on her upper and lower extremities. Histopathologically, the small-to medium-sized arteries in the deep dermis and subcutis showed necrotizing vasculitis with cellular infiltration, suggesting cutaneous polyarteritis nodosa. The serum levels of inflammatory markers normalized with aspirin 100mg/day and prednisolone 10mg/day within 2 months, and there was no other skin or organ involvement over 18 months of follow up. However, serious refractory skin depressions and pigmentation remained after two years of treatment. This suggests the importance of early and aggressive therapy for cutaneous polyarteritis nodosa to prevent unsightly skin sequel, as well as control of disease activity.

Cutaneous polyarteritis nodosa

Polyarteritis nodosa is a rare vasculitis in children characterized by necrotizing inflammation in small and medium size arteries. It is classified into systemic and cutaneous PAN according to the presence of systemic symptoms or visceral involvement. We describe the case of a 14-year-old girl with cutaneous Polyarteritis nodosa with an atypical clinical presentation.

Manifestaciones clínicas de la granulomatosis de Wegener y la poliangeítis microscópica en Santiago-Chile, 1990-2001 / Clinical features of Wegener granulomatosis and microscopic polyangiitis in Chilean patients

Background: Systemic vasculitis are a group of heterogeneous diseases characterized by inflammation and necrosis of blood vessel walls. The etiology is not known, but geographic and environmental factors are implicated. Aim: To describe the clinical features of microscopic polyangiitis (MPA) and Wegener's granulomatosis (WG) in a Chilean cohort of patients. Patients and methods: Retrospective review of the medical records of 123 patients with the diagnosis of systemic vasculitis (65 MPA and 58 WG), seen from 1990 to 2001. The diagnosis were made based on the American College of Rheumatology and Chapel Hill criteria. Results: The mean follow-up for MPA was 15 months (1-120) and for WG, 20 months (1-120). The median age (years) at diagnosis for MPA was 61 (19-82) and WG 50 (20-82). Gender distribution was similar in both groups (male: 68percent and 57percent respectively).The main clinical features in the MPA group were renal involvement (68percent), peripheral nervous system involvement (57percent), pulmonary hemorrhage (28percent), and skin disease (32percent). In the WG group were alveolar hemorrhage (62percent), renal involvement (78percent), paranasal sinus involvement (57percent), and ocular disease (26percent). In both, creatinine levels above 2.0 mg/dl were associated with a higher mortality (p< 0.01). ANCA by immunofluorescence was performed in 56 MPA patients (75percent had pANCA, 4percent had cANCA and 21percent were ANCA negative) and in 55 WG patients (17percent had pANCA, 79percent had cANCA and 4percent were ANCA negative). Global mortality was 18percent and 17percent respectively, and the most common causes of death were infections. Conclusions: The clinical features of our patients are similar to other published data. In our WG and MPA patients the main predictor for death was a serum creatinine above 2 mg/dl.

Idiopathic canine polyarteritis in control beagle dogs from toxicity studies

It is sometimes difficult to assess the relevance of polyarteritis with treatment-related lesions in dog toxicity studies, as number of dogs used in a toxicity study is small and the lesions are similar to those seen in spontaneous diseases. This report is intended to establish a general profile of idiopathic canine polyarteritis in beagle dogs. Data from a total of 40 dog studies including 4-, 13- or 52- weeks studies conducted between 1990 and 2003 at Huntingdon Life Sciences, UK, were collected and analysed. There was no death by this disease and also no prominent clinical signs related to this disease. Histologically, males tended to develop polyarteritis more frequently than in females and epididymis is the most probable tissues, followed by thymus and heart. Dogs in two studies showed higher incidences of these lesions, whereas animals in the other studies did not exhibited, suggesting that genetic predilection plays an important role in this disease.

Isolated necrotising arteritis of the cervix and myometrium: a much neglected but puzzling entity.

Arteritis of the uterine cervix and corpus described here was an incidental finding at the routine histopathological examination of the hysterectomy specimen resected from a 62 year old female who underwent laparotomy for twisted ovarian cyst. Investigations and eleven months of follow up without any specific treatment for arteritis, have shown no systemic involvement. This case highlights that a knowledge of such isolated arteritis is of importance to the physician to avoid misdiagnosing it as polyarteritis nodosa and treat with systemic steroids.

Facts about artefacts in diagnostic pathology.

Literal meaning of artefact given by 'Oxford Advanced Learner Dictionary' is 'a thing made by people'. In medical science 'the fact' is not true; but we observe routinely is called artefact. We face various types of artefacts in daily reporting of pathology specimen. Many times artefacts hinder the actual diagnosis. The artefacts i. e. fixation artefact, processing artefact, staining artefact, mounting artefact, air bubbles etc. can cause difficulty in diagnosis and a pathologist should be trained to identify those artefacts.

Poliarterite nodosa: consideraçöes em torno de um caso com verificaçäo à necropsia / Nodular polyarteritis: considerations about a case with verification by necropsy

Por meio da necropsia é apresentado um caso com estudo clínico e anátomo-patológico. Enfatiza-se sobretudo o estudo histopatológico das artérias de médio calibre do epicardio e dos rins, a fim de encontrar os aspectos típicos desta entidade. Säo feitas consideraçöes sobre os diagnósticos clínicos e anátomo-patológicos, acentuando as dificuldades para o estabelecimento do diagnóstico clínico, razäo por que, na maioria das vezes, o diagnóstico é feito pela necropsia.

Poliarteritis nodosa cutánea benigna en un caso / Benign cutaneous polyarteritis nodosa in 1 case

Cutaneous polyarteritis nodosa is a benign form of the disease with cutaneous involvement and occasional systemic manifestations. We report a 51 years old man presenting with malaise, arthralgia, myalgia and nodular, erythematous and painful skin lesions in both legs. The pathological study of these lesions showed a polyarteritis. During hospital stay there was a mild remission of the disease without treatment, but it reappearence during follow lead to the use of prednisone