Signs Of Vasculitis Research Articles

Nicolau syndrome following intramuscular diclofenac injection

Sir, Nicolau syndrome (NS), also known as embolia cutis medicomentosa and livedo-like dermatitis, is a rare cutaneous adverse reaction at the site of intramuscular, intra-articular or, rarely, subcutaneous injection of particular drugs. NS was first described in patients treated for syphilis with bismuth salts in 1925.[1] It has been reported in the literature with the administration of various other drugs such as penicillin, non-steroidal anti-inflammatory drugs, local anesthetics, vitamin K, triple DTB vaccination, and corticosteroids.[2–5] Although its pathogenesis is not well understood, the pathophysiology of NS probably involves intra-arterial and/or para-arterial injection and arterial embolism of viscous suspensions of drugs meant for intramuscular, intravenous, or intra-articular injection, followed by acute vasospasm.[2] We report the case of a 77-year-old female patient who presented a painful ecchymotic plaque on the right buttock. A week ago, she had been given an intramuscular diclofenac injection, and livid discoloration appeared around the injected area. Over the following days, erythema, edema, and painful hemorrhagic blisters developed around the ecchymosis. On physical examination, a 5 × 4-cm-size, well-defined ecchymotic plaque surrounded by a livedoid, reticular erythematous patch with ruptured blister particles was observed on the right buttock [Figure 1]. General examination was normal, and blood testing revealed a mildly raised erythrocyte sedimentation rate (ESR: 36). Doppler ultrasonographic evaluations for the right lower limb were normal. Histopathologic examination revealed focal necrosis areas of the epidermis, and perivascular eosinophilic infiltrate with extravasation of erythrocytes, no sign of vasculitis was detected.Figure 1: Presenting skin lesionThe differential diagnosis of NS includes cutaneous cholesterol emboli, vasculitis, and cutaneous embolization of cardiac myxoma. Cholesterol emboli usually occurs in the elderly patients with severe atherosclerotic disease and after endovascular manipulation. Left atrial myxomas can cause cutaneous emboli, usually to the acral sites and accompanied by cardiopulmonary symptoms such as chest pain and dyspnea.[6] Our patient did not have any other comorbid conditions such as cardiovascular disease or diabetes. Clinically and histopathologically, the patient was diagnosed as having Nicolau syndrome and treated conservatively with local wound care. Within the following weeks, a necrotic ulcer developed [Figure 2] and finally an atrophic scar remained at the site of injection at three-month follow up.Figure 2: Clinical appearance of the lesion within the following weeksThere is no specific therapy for NS. Treatment depends on the extent of the necrosis, and ranges from topical to surgical. Conservative treatment with debridement, pain control (analgesics), and dressings is the mainstay of therapy, especially for limited cases. Tissue damage may be reversible in the acute phase of NS. Use of vasoactive agents such as subcutaneous heparin and oral pentoxifylline has been recognized as beneficial. Surgical intervention is rarely required.[2] Using of hyperbaric oxygen therapy in selected cases has been reported in the literature.[7] Although NS is an uncommon cutaneous adverse reaction, the symptoms are dramatic, and this rare complication can cause severe anxiety to the patient. Healthcare personnel should be aware of this condition and take adequate precautions. A sufficiently long needle for an obese patient, the Z-track method of intramuscular injection, injection only after aspiration with a syringe, and an injection in the upper outer quadrant of the gluteal region which has few large blood vessels, can minimize or prevent Nicolau syndrome.

Isolated necrotising arteritis of the appendix in a 17-year-old male

Here we repo rt a case of necrotising arteritis of the appendix in a 17-year-old male presenting with right lower quadrant pain. The appendix was macroscopically normal. Histological examination showed fibrous obliteration of the tip with necrotising vasculitis involving small to medium-sized arteries comprised of fibrinoid necrosis and neutrophilic infiltrate, karyorrhexis and cuffing by histiocytes and lymphocytes. No acute appendicitis was found. Isolated necrotising vasculitis of the appendix is an uncommon finding of long debated relatedness to systemic vasculitic disorders. Our patient showed no clinical signs of vasculitis and tested negative for pANCA, cANCA, ANA and ENA. A risk of systemic vasculitis approaching 30% has been reported in some studies,¹ however these are limited by study size. Polyarteritis nodosa is the most commonly associated systemic vasculitic disease. Careful histological examination is required to identify vasculitis. Given the rarity of the condition, we suggest routine follow-up of all patients by clinical and serological screening for systemic vasculitis. The question of duration of screening in a young patient with no significant family history is difficult to answer.

Cryoglobulinemic vasculitis in systemic sclerosis successfully treated with mycophenolate mofetil

Cryoglobulinemic vasculitis is extremely rare in patients with systemic sclerosis (SSc). So far, only two cases of cryoglobulinemic vasculitis in SSc were described in the literature. This report is about a patient with SSc and secondary Sjőgren's syndrome, who developed typical clinical features of small-vessel vasculitis, including arthritis, purpura, microhaematuria, gangrene of fingers, and toes and myocardial ischemia, in the presence of mixed cryoglobulinemia, ANA, rheumatoid factor, and anti-SSA/Ro antibodies. Symptoms and signs of vasculitis worsened despite initial treatment with corticosteroids and cyclophosphamide, but improved significantly when mycophenolate mofetil was used instead cyclophosphamide.

Usefulness of (18)F-FDG PET/CT in Evaluating Disease Activity at Different Times in a Patient With Chronic Periaortitis.

We report the case of a 53-year-old man with a chronic periaortitis (CP) in whom Fluorine-18-Fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) has been useful in assessing the disease extent at diagnosis, in evaluating the treatment response, in diagnosing the relapse, and in monitoring the disease activity during follow-up (Fig. 1). This case highlights the usefulness of FDG-PET/CT at different times in patients with CP. Fig. 1 A 53-year-old man was admitted to our hospital for a suspected chronic periaortitis (CP) based on clinical (abdominal pain), laboratory data (increased serological inflammatory markers, such as erythrocyte sedimentation rate [ESR] and C reactive protein ... CP is a rare inflammatory disorder that affects the abdominal aorta and the retroperitoneum [1–3]. Imaging procedures are essential to diagnose and monitor the disease course [1–3]. Ultrasonography may be used as first-line screening test, and is particularly useful to monitor patients with hydronephrosis and aortic aneurysms. However, CT or magnetic resonance imaging (MRI) of the abdomen are currently considered the investigations of choice to diagnose CP [1–3]. In active CP, the periaortic mass usually enhances on CT and MRI. Both these methods can be used to monitor the disease course of CP and to evaluate response to treatment. A note of caution: signs of vasculitis seen on MRI and CT may persist for some time, despite achievement of clinical remission. A further limitation of abdominal CT and MRI is that neither technique can detect vasculitis in vessels other than the abdominal aorta, which has been described in 43 % of patients with CP [1]. FDG-PET/CT has been proposed as useful tool in inflammatory diseases, such as aortitis, because metabolic changes assessed by FDG-PET usually precede morphological changes assessed by conventional imaging methods [4–8]. Our case highlights the usefulness of FDG-PET/CT at different times in patients with CP. This method may be useful in assessing disease activity and extent at diagnosis, in evaluating the treatment response, in diagnosing the relapse, and in monitoring the disease during follow-up.

Sweet's syndrome: clinicopathological features of patients treated from 1997 to 2009 at Cassiano Antonio Moraes University Hospital - Vitoria (Espirito Santo)

Sweet's syndrome or acute febrile neutrophilic dermatosis is rare in Brazil. It is clinically characterized by painful erythematous nodules, papules or plaques that occur mainly on the neck and upper limbs. Its cause may be unknown (idiopathic form) or it may be associated with malignancies, usually hematologic, or drugs. The authors describe 16 cases of the syndrome. The median age was 36 years, and all patients were white and presented solid lesions, predominantly on the upper limbs and trunk. Histopathological examination of the dermis of all patients revealed predominance of moderate to intense, superficial and deep, diffuse inflammatory infiltrate, mainly consisting of polymorphonuclear neutrophils, with leukocytoclasia. It also revealed changes in the epidermis and hypodermis (neutrophilic hypodermitis), but with no signs of vasculitis in most patients, which was not considered an important finding for diagnosis. Presence of exocytosis of neutrophils was common, favoring the diagnosis of Sweet's syndrome when accompanied by diffuse interstitial neutrophilic dermatitis.

Birdshot-like chorioretinopathy in common variable immunodeficiency.

To describe a patient with ocular manifestations secondary to common variable immunodeficiency. A 17-year-old adolescent girl presented with asymptomatic bilateral optic disk edema and chorioretinal infiltrates. These findings were noted during routine follow-up visits for hydroxychloroquine use for lymphoid interstitial pneumonia. She underwent a full ophthalmologic and systemic work-up. At the age of 13, the patient was diagnosed with common variable immunodeficiency with splenomegaly, lymphadenopathy, and lymphoid interstitial pneumonia. Progressive generalized lymphadenopathy developed thereafter. Since the diagnosis of common variable immunodeficiency, she received monthly intravenous immunoglobulin, systemic corticosteroids and later on, oral hydroxychloroquine. Annual routine screening visits to exclude retinal hydroxychloroquine toxicity were unremarkable until age 17. Unaided visual acuity was 6/5 in both eyes. Slit-lamp examination was normal, whereas fundoscopy revealed bilateral optic disk edema and birdshot-like chorioretinal infiltrates, without signs of vitritis. A bilateral hot disk was seen on fluorescein angiography, but there were no signs of vasculitis. Indocyanine green angiography showed macular and midperipheral choroidal granulomas. A brain magnetic resonance imaging scan revealed no signs of raised intracranial pressure. Human leukocyte antigen-typing was negative for HLA-A29, making birdshot chorioretinopathy highly unlikely. The results of a work-up for sarcoidosis were not typical of that disease. During the past months, a remarkable episode of lacrimal gland swelling with spontaneous recovery was noted. We describe a case of ocular manifestations of common variable immunodeficiency, including asymptomatic bilateral chorioretinal granulomas mimicking birdshot chorioretinopathy, combined with optic nerve sheath infiltration, and lacrimal gland involvement.

A retrospective analysis of histopathology of 64 cases of lepra reactions

Background:Lepra reactions are not always diagnosable under the microscope. We analyzed skin histopathology in 64 cases of lepra reaction.Aim:To make detailed observations on histopathologic features of type 1 and type 2 lepra reaction (erythema nodosum leprosum, ENL).Materials and Methods:In this retrospective study, we included 64 patients diagnosed during a 3-year period as lepra reaction based on clinico-pathological co-relation.Results:Out of the 64 patients, 22 were of type 1 reaction and 42 of ENL. The most consistent finding in type 1 reaction was papillary dermal edema (86%) followed by pyknosis of lymphocytes (77%) and intercellular edema within granuloma (73%). Surprisingly, folliculotropism of lymphocytes was seen in 55% and subcutaneous infiltration in 36%. In ENL, the most common finding was presence of neutrophils within the granuloma (100%), followed by leukocytoclasia (81%), papillary dermal edema (81%), and neutrophilic panniculitis (69%). Fibrin in the vessel wall or/and granulomas was noted in only 38% while fibrin thrombi in the vessel walls were seen in only 12% of cases.Conclusion:Infiltration of macrophage granulomas by neutrophils is a reliable sign of ENL. Classical signs of vasculitis are not always present in ENL. Folliculotropism and lymphocytic panniculitis were frequent in type 1 reactions while neutrophilic panniculitis was common with ENL.

Recurrent ischemic strokes in a young celiac woman withMTHFRgene mutation

Celiac disease (CD) is frequently associated with neurological disorders, but very few reports concern the association with ischemic stroke. A 26-year-old woman affected by CD with secondary amenorrhea, carrier of a homozygous 5,10-methylenetetrahydrofolate reductase mutation with hyperhomocysteinemia, was affected by two occipital ischemic strokes within a period of 5 mo. At the time of the second stroke, while she was being treated with folic acid, acetylsalicylic acid and a gluten-free diet, she had left hemianopsia, left hemiparesthesias, and gait imbalance. Brain magnetic resonance imaging showed a subacute right occipital ischemic lesion, which was extended to the dorsal region of the right thalamus and the ipsilateral thalamo-capsular junction. Antitransglutaminase and deamidated gliadin peptide antibodies were no longer present, while antinuclear antibodies, antineuronal antibodies and immune circulating complexes were only slightly elevated. Since the patient was taking folic acid, her homocysteine ​​levels were almost normal and apparently not sufficient alone to explain the clinical event. A conventional cerebral angiography showed no signs of vasculitis. Finally, rare causes of occipital stroke in young patients, such as Fabry's disease and mitochondrial myopathy, encephalomyopathy, lactic acidosis and stroke-like symptoms, were also excluded by appropriate tests. Thus, the most probable cause for the recurrent strokes in this young woman remained CD, although the mechanisms involved are still unknown. The two main hypotheses concern malabsorption (with consequent deficiency of vitamins known to exert neurotrophic and neuroprotective effects) and immune-mediated mechanisms. CD should be kept in mind in the differential diagnosis of ischemic stroke in young patients.

Teaching Neuro Images : Cochleitis

A 60-year-old woman with history of multineuritis and hepatitis C virus (HCV) infection suddenly developed bilateral asymmetric sensorineural hearing loss (SNHL). Laboratory panel found a HCV load of 2,031,900 UI/mL and a type II mixed cryoglobulinemia. Brain MRI revealed asymmetric contrast enhancement of right cochlea (figure 1). Sural nerve biopsy showed indirect signs of vasculitis (figure 2). Many …

Hbv-Related Liver Disease in Renal Insufficiency: Successful Antiviral Therapy with Entecavir

Hepatitis B virus (HBV) infection persists among patients with renal insufficiency in the industrialized world. A variety of therapeutic options are now available for the treatment of chronic hepatitis B infection, including potent new nucleos(t)ide analogs, along with standard and pegylated interferon. We report on a patient with aggressive hepatitis B and renal insufficiency who was successfully treated with nucleoside analogs (mostly entecavir monotherapy) for two years. He received intense immunosuppression for severe myopathy of lower limbs, probably related to vasculitis. HBV DNA (at the beginning, > 1 x 10(8) IU/mL) was no longer detectable in serum after a few months of antiviral therapy, while HBeAg and HBsAg seroconversion occurred with ALT normalization. Clinical signs of vasculitis disappeared. Five months after discontinuation of entecavir therapy, he remained HBsAg negative with detectable anti-HBs antibody in serum. We conclude that nucleos(t)ide analogs can offer excellent response in selected patients with renal insufficiency and hepatitis B-related liver disease. Prospective, controlled clinical trials are needed to confirm these encouraging results.

Fibronectin gene polymorphisms and clinical manifestations of mixed cryoglobulinemic syndrome: increased risk of lymphoma associated to MspI DD and HaeIII AA genotypes

To analyse FN gene polymorphisms in type II mixed cryoglobulinemic syndrome (MCsn), an immune-complex mediated systemic vasculitis linked to hepatitis C virus (HCV) infection and characterized by rheumatoid factor (RF) positive B-cell proliferation at high risk for the progression into non Hogkin's lymphoma (NHL). Samples from eighty-one patients, with MCsn (type II serum cryoglobulins and clinical signs of vasculitis were studied. Sixty-five (65/81, 80.3%) patients were HCV-positive. Twenty-one (25.9%) patients had developed a B-cell NHL during the course of MCsn. Seventy-two patients with HCV-negative and MC-unrelated NHL and 110 healthy blood donors (HBDs) were taken as controls. HaeIIIb and MspI FN gene polymorphisms were analysed by ELISA, whenever possible. HaeIIIb and MspI allele and genotypic frequencies did not differ between MCsn patients and HBDs. Of note, the DD-MspI allele and genotype frequencies did not differ between MCsn patients and HBDs. Of note, the DD-MspI (OR = 5.56; DI = 1.67-18.51, p = 0.0046) and the AA-HaeIIIb (OR = 5.54, CI = 1.64- 18.76, p = 0.0066) homozygosis appeared significantly and independently associated with the development of B-cell NHL in MCsn patients, with the HaeIIIbA allele possibly conferring an increased risk of NHL in the general population (OR = 1.72, CI = 1.128-2.635, p = 0.0133). In contrast, the major vasculitic manifestations, such as peripheral neuropathy, skin ulcers and glomerulonephritis tended to be associated with the counterpart MspI C allele. No association between FN plasma levels and FN genotypes was found. Genotyping for MspI and HaeIIIb FN gene polymorphisms may be clinically relevant to define the predisposition to the major clinical manifestations in MCsn.

Streptococcus pneumoniae Meningoencephalitis With Bilateral Basal Ganglia Necrosis

Streptococcus pneumoniae (S pneumoniae) is a common cause of bacterial meningitis, frequently leading to death or severe neurological impairment. We report an exceptional case of a 4-month-old boy with meningitis caused by S pneumoniae. Computed tomography (CT) and magnetic resonance imaging (MRI) examinations of the brain showed bilateral symmetrical necrosis involving the lentiform and caudate nuclei, as well as the thalamus. T1-weighted MR images showed patchy areas of increased signal intensity, consistent with hemorrhagic transformation of the lesions. Autopsy revealed widespread necrosis of the basal ganglia with clear signs of vasculitis. Severe complications of S pneumoniae meningoencephalitis are known in infants but to our knowledge, such lesions in the basal ganglia have only been reported thrice in adults and never in children.

Rituximab in c-ANCA associated vasculitic polyneuropathy complicated by local peritonitis

A 70-year-old woman presented with progressive weakness over 3 weeks and paresthesia of the lower extremities. Except for a recent history of recurrent respiratory tract infections, she had been healthy previously. Within hours after hospital admission, paresis progressed markedly, and she required intensive care. Physical examination showed sensory-motor polyneuropathy with tetraplegia and symmetrical distal sensory impairment of all extremities, no cranial nerve abnormalities nor autonomous dysregulation. Because of involvement of the diaphragm the patient was intubated and ventilated. The clinical presentation was suggestive of Guillain–Barre syndrome (GBS). CSF examination revealed 92 WBC/ll, 33,700 erythrocytes/ll, glucose 69 mg/dl, lactate 35.4 mg/dl and protein 181 mg/dl. Electrophysiology was consistent with a sensorymotor polyneuropathy with axonal affection. A cranial MRI demonstrated central nervous system involvement (Fig. 1g–i). Laboratory testings showed an elevated c-antineutrophil cytoplasmic antibodies (c-ANCA)-titer (168 U/ml) and slightly elevated creatinine. In synopsis with the patient’s history of recurrent respiratory infections, Wegener’s granulomatosis with vasculitic polyneuropathy and central nervous system involvement was suspected and confirmed by sural nerve biopsy (Fig. 1a–f), as well as skin , and nasopharyngeal mucosa biopsy (not shown). High-dose steroids, plasmapheresis, and intravenous immunoglobulins were sequentially administered without improving the patient’s symptoms. Then a severe lower gastro-intestinal bleeding, which eventually required surgical removal of the ileocecum complicated the clinical course. Histology, it revealed no signs of vasculitis, but a disseminated cryptitis of the colon. Subsequently, the patient developed a localized peritonitis due to abdominal wound healing disturbance and dehiscence, warranting multiple surgical revisions. Meanwhile, the patient’s neurological and pulmonary status remained unchanged. Because therapy-escalation seemed mandatory, administration of rituximab (dose: 375 mg/m in 4 weekly courses) was preferred to cyclophosphamide, which is described to worsen wound healing [1]. Remarkably, under rituximab, the abdominal infection did not recur and the patient’s condition improved substantially. Ten weeks after admission, the patient could be weaned off the ventilator and transferred to the general ward. Within the following weeks, muscle strength in the upper, and later on in the lower extremities, recurred and the patient was transferred to rehabilitation. The patient was able to touch her nose with the left hand and to lift up her right hand against gravity. Both legs could be elevated against gravity but she was still unable to walk. The neurological improvement was quantified using the Wegener’s J. Witsch (&) J. Braun G. Trendelenburg Department of Neurology, Charite University Medicine, Campus Virchow Klinikum, Augustenburger Platz 1, 13353 Berlin, Germany e-mail: Jens.Witsch@charite.de

Pyoderma gangrenosum associated with renal carcinoma

Pyoderma gangrenosum associated with renal carcinoma

Crioglobulinemia tipo I de desenlace fatal

Type I cryoglobulinemia, a condition associated with lymphoproliferative disorders, is caused by monoclonal immunoglobulins that precipitate at low temperatures. It mostly involves the skin and pathology study shows no signs of vasculitis. Management is usually based on immunosuppressive drugs associated with plasmapheresis for severe disease. The use of rituximab has recently been described for resistant cases. We present an unusual case of long-standing type I cryoglobulinemia associated with a monoclonal gammopathy of unknown significance. The patient developed extremely severe skin lesions with histological signs of vasculitis. The patient died due to the onset of noncutaneous manifestations of the cryoglobulinemia and complications of the immunosuppressive treatment.

Photodistributed Telangiectasia Induced by Amlodipine

Calcium channel blockers are widely used antihypertensive drugs, which are uncommonly associated with cutaneous reactions, such as pruritus, urticaria, or alopecia. Photosensitivity presenting with telangiectasia has rarely been described. We present here a case of photodistributed telangiectasia induced clinically by amlodipine and histologically by enlarged capillaries in the upper dermis without signs of vasculitis.

Hepatitis C virus: A rare manifestation - Remitting relapsing central and peripheral demyelination

The most frequent neurologic manifestations of hepatitis C virus infection include peripheral neuropathy axonal type and central nervous system (CNS) vasculitis. Affected patients usually have cryoglobulinemia and other signs of vasculitis. Demyelinating lesions, both central and peripheral are rarely described. We present a case of simultaneous peripheral nervous system and CNS demyelination that comes in relapsing episodes, with negative cryoglobulins.

A Case of a False-Positive Anti-Myeloperoxidase Antibody ELISA in a Patient with Hypothyroidism

We present a case of a false-positive anti-myeloperoxidase (MPO) antibody result on an ELISA in a patient with anti-thyroid microsomal antibody (TMA)-positive hypothyroidism. A 41-year-old woman presented with dyspnea on exertion. The initial evaluation revealed pericardial effusion associated with hypothyroidism. In addition, microscopic hematuria with normal renal function and positive cytoplasmic anti-neutrophil cytoplasmic antibodies (c-ANCA) on immunofluorescent assay were found. In further evaluation, elevated anti-TMA and MPO antibodies by ELISA. While no definite signs of vasculitis were present, the clinical state improved with thyroid hormone replacement and diuretics. Anti-MPO antibody was still positive in the follow-up tests, and microscopic hematuria persisted. On the basis of previous reports that thyroid peroxidase and MPO molecules contain cross-reactive epitopes that are exposed in denaturated molecules, we suggest that in a patient with anti-TMA-positive hypothyroidism, anti-MPO antibody might also be positive on ELISA without clinical signs of vasculitis.

Paquimeningitis craneal hipertrófica idiopática: reporte de caso

Cranial idiopathic hypertrophic pachymeningitis is a rare disorder characterized by chronic inflammation causing thickening of the dura mater. Headache, multiple cranial neuropathy and magnetic resonance images showing GD-DTPA-enhanced thickened dura can aid diagnosis. We report the autopsy case of a 69-year-old man with chronic headache and visual loss due to optic nerve damage developing over a 3-month period, who was diagnosed with hypertrophic pachymeningitis. Cerebrospinal fluid showed inflammatory changes. The sample of hypertrophied dura mater revealed chronic granulomatous inflammation without signs of vasculitis or infectious or neoplastic processes. Idiopathic hypertrophic pachymeningitis is an exclusion diagnosis. Exhaustive tests (both bacteriological and histopathological) to identify the cause of dural thickening in our patient proved unsuccessful.

Churg-Strauss syndrome following cessation of allergic desensitization vaccination: a case report

IntroductionChurg-Strauss syndrome is a vasculitis of medium to small sized vessels. Diagnosis is mainly clinical with findings of asthma, eosinophilia, rhinosinusitis and signs of vasculitis in major organs.Case presentationWe present a case of a 19-year-old Persian male who developed signs and symptoms of this syndrome related to hyposensitization treatments for allergy control.ConclusionsNo unifying etiology for the disease can be presented as it is found associated with environmental factors, medications, infections and is even considered a variant of asthma with predisposition to vasculitic involvement. Therefore, it is important to recognize this disease and be aware of underdiagnosis because of emphasis on pathologic evidence. Here, we present a case of allergic desensitization causing Churg-Strauss syndrome in the absence of other known factors.