Presentation
We review the clinical and pathological findings of ALCL and present this case to highlight a subtle diagnostic clue in assessing recurrence of cutaneous lymphoma. [ncbi.nlm.nih.gov]
Entire Body System
- Fever
Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disease, which is characterized by recurrent and paroxysmal fever, peritonitis, arthritis, myalgia, and skin rashes. [ncbi.nlm.nih.gov]
- Weight Loss
She had experienced high fever, weight loss, polyarthralgia, myalgia, polyneuropathy and hallucinations for 3 years before admission. [ncbi.nlm.nih.gov]
Common signs and symptoms of necrotizing vasculitis include: Chills, Fatigue, Fever Weight loss Anemia, Leukocytosis Skin discoloration with ulcers Kidney damage Tissue death ( necrosis) Muscle and joint pain Declined brain functioning How is Necrotizing [dovemed.com]
Fever, chills, fatigue, arthritis, or weight loss may be the only symptoms at first. However, symptoms may be in almost any part of the body. [medlineplus.gov]
- Chills
Common signs and symptoms of necrotizing vasculitis include: Chills, Fatigue, Fever Weight loss Anemia, Leukocytosis Skin discoloration with ulcers Kidney damage Tissue death ( necrosis) Muscle and joint pain Declined brain functioning How is Necrotizing [dovemed.com]
Fever, chills, fatigue, arthritis, or weight loss may be the only symptoms at first. However, symptoms may be in almost any part of the body. [medlineplus.gov]
Symptoms Fever, chills, fatigue, arthritis, or weight loss may be the only symptoms at first. However, symptoms may be in almost any part of the body. [mountsinai.org]
These include: chills fatigue fever weight loss Other early symptoms are only detectable through a blood test. These include leukocytosis, which involves having a high number of white blood cells (WBCs) and anemia. [healthline.com]
Systemically the patient denied any fevers, chills, chest pain, shortness of breath, abdominal pain, hematuria or joint complaints. Her past medical history was significant for Graves’ disease, which was being followed by her endocrinologist. [westjem.com]
- Malaise
See: illustration ; autoimmune disease ; immune complex Symptoms Although fever, pain, and malaise are common, the inflammatory changes of the blood vessels are seen primarily through the signs and symptoms associated with the organ or tissues involved [medical-dictionary.thefreedictionary.com]
Signs of PTU-induced vasculitis include anemia (seen in the majority of patients), skin lesions (ulcers, purpura, subcutaneous nodules, and erythema nodosum-like and erythema multiforme-like lesions), arthralgia, fever, malaise, lymphadenopathy, hematuria [westjem.com]
Patients can present to the family physician with nonspecific signs or symptoms (e.g., fever, rash, myalgia, arthralgia, malaise, weight loss) or to the emergency department with life-threatening features (e.g., massive hemoptysis, renal failure). [aafp.org]
- Falling
Wegener's Foundation, Inc. 3705 South George Mason Drive, Suite 1813 South, Falls Church, VA 22041. (703) 931-5852. vasculitis [ vas″ku-li´tis ] hypersensitivity vasculitis a group of systemic necrotizing vasculitides thought to represent hypersensitivity [medical-dictionary.thefreedictionary.com]
Although a punch or shave biopsy usually suffices in suspected small vessel vasculitis (SVV), it falls short in the other two categories. [ijdpdd.com]
The cyclophosphamide dose is reduced only if the peripheral leukocyte count drops below 3000/3 x 109 /L or the absolute neutrophil count falls below 1500/1.5 x 109/L. [lupusinternational.com]
Respiratoric
- Hoarseness
Swallowing difficulty Speech impairment Movement difficulty Lungs and respiratory tract: Cough Shortness of breath Sinus congestion and pain Coughing up blood or bleeding from the nose Other symptoms include: Abdominal pain Blood in the urine or stools Hoarseness [medlineplus.gov]
Brochoscopy with tracheobronchial mucosal biopsy may be useful in evaluating lower airway symptoms such as cough, dyspnea, wheezing, or hoarseness. [lupusinternational.com]
Gastrointestinal
- Abdominal Pain
One month later, she began experiencing high fevers, headache, myalgia, neck stiffness, and abdominal pain as well as bilateral blurred vision from APMPPE. [ncbi.nlm.nih.gov]
Lungs and respiratory tract: Cough Shortness of breath Sinus congestion and pain Coughing up blood or bleeding from the nose Other symptoms include: Abdominal pain Blood in the urine or stools Hoarseness or changing voice Chest pain from damage of the [medlineplus.gov]
She visited our hospital with the chief complaint of lower abdominal pain. Since free air in the abdomen was detected by abdominal X-ray and CT scanning, perforation in the lower digestive tract was diagnosed, and laparotomy was performed. [ci.nii.ac.jp]
Family history was remarkable for his late father who had had abdominal pain and arthralgia. Two of his children had recurrent febrile episodes. [ard.bmj.com]
Musculoskeletal
- Arthralgia
Within 14 days of interferon-alpha therapy (3 x 3 million IU/ml/week), purpuric skin lesions disappeared as well as fatigue and arthralgia; 9 months after initiation of therapy, liver enzyme values were nearly normal despite persistence of HCV RNA tested [ncbi.nlm.nih.gov]
myalgia Diagnosis,* disease monitoring Complement (C3 and C4) Arthralgia, myalgia Disease monitoring Cryoglobulins Purpura, arthritis, hepatitis C Diagnosis of cryoglobulinemic vasculitis Immunoglobulins and protein electrophoresis Arthralgia, bone [aafp.org]
Myalgias with areas of focal ischemic myositis and arthralgias are common. Affected muscles are tender and may be weak. Arthritis may occur. [msdmanuals.com]
Leukocytoclastic vasculitis: neutrophilic inflammation with fibrinoid necrosis and fragmented neutrophilic nuclei (leukocytoclasis); it presents as purpuric palpable lesions of lower legs; systemic cases are associated with Henoch-Schonlein pupura (fever, arthralgias [pathologyoutlines.com]
Family history was remarkable for his late father who had had abdominal pain and arthralgia. Two of his children had recurrent febrile episodes. [ard.bmj.com]
- Muscle Weakness
Neurologic examination revealed muscle weakness and atrophy of the legs, more marked in the proximal muscles, and deep-tendon reflexes were diminished. Blood cell counts showed mild leukocytosis and thrombocytosis. [neurology.org]
Muscle weakness Brain and nervous system: Pain, numbness, tingling in an arm, leg, or other body area Weakness of an arm, leg, or other body area Pupils that are different sizes Eyelid drooping Swallowing difficulty Speech impairment Movement difficulty [medlineplus.gov]
If nerves suffer loss of blood supply, there may initially be an abnormal sensation followed by a loss of sensation or muscle weakness. Vasculitis in the brain may cause a stroke, or in the heart, may result in a heart attack. [webmd.com]
- Leg Pain
Skin: Red or purple colored bumps on the legs, hands or other parts of the body Bluish color to the fingers and toes Signs of tissue death due to lack of oxygen such as pain, redness, and ulcers that do not heal Muscles and joints: Joint pain Leg pain [medlineplus.gov]
In February 2014, after three months of therapy, patient complained left leg pain and edema. Clinical examination showed several harsh and painful skin lesions on palpation ( Figure 1A,B ); AFP was 3,179 ng/mL, doubled compared to baseline. [jgo.amegroups.com]
- Osteoporosis
Patients can experience considerable treatment-related toxicity, especially infection from immunosuppressive therapy and adverse effects from steroids (e.g., osteoporosis, diabetes mellitus, cataract). [aafp.org]
Unless contraindicated, 400 IU of vitamin D and 1000 to 1500 mg of elemental calcium can be given daily to retard the development of glucocorticoid-induced osteoporosis. [lupusinternational.com]
Skin
- Skin Ulcer
Cutaneous: Livedo reticularis, skin ulcers, tender erythematous nodules, bullous or vesicular eruptions, infarction and gangrene of fingers or toes, or a combination may occur. [msdmanuals.com]
It can produce deep dermal ulcers, localized skin infarction, ischemia or necrosis of the fingertips, scleritis, and inflammation of multiple nerves (mononeuritis multiplex). vasculitis Widespread inflammation of blood vessels occurring as the principal [medical-dictionary.thefreedictionary.com]
Infections of chronic, deep skin ulcers have led to death from sepsis. Some investigators have postulated that HCV-related type II MC is a benign of low-grade B-cell lymphoproliferative disease. [lupusinternational.com]
Face, Head & Neck
- Epistaxis
Thirteen years later, he inadvertently received intravenous ciprofloxacin and developed severe headache and epistaxis. Serologic testing at that time revealed elevated titers of PR3-ANCA. [scopemed.org]
Case report A 38-years-old Gambian man was admitted for necrotic and infiltrated purpura of acral distribution (limbs, ears and nose), which was associated with multiple subcutaneous inflammatory nodules, epistaxis, polyarthralgia, fever and a poor general [ijams.kibanresearchpublications.com]
Upper airway disease with sinusitis, otitis, epistaxis, and subglottic and/or tracheal stenosis occurs in 70-95% of patients. The presence of ulcerating or destructive lesions is highly suggestive. [clinicaladvisor.com]
Urogenital
- Oliguria
Renal: If small- and medium-sized arteries in the kidneys are affected, patients may have hypertension, oliguria, uremia, and a nonspecific urinary sediment with hematuria, proteinuria, and no cellular casts. Hypertension may worsen rapidly. [msdmanuals.com]
Neurologic
- Polyneuropathy
She had experienced high fever, weight loss, polyarthralgia, myalgia, polyneuropathy and hallucinations for 3 years before admission. [ncbi.nlm.nih.gov]
[…] motor polyneuropathy 0 case Table 2. [oatext.com]
Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is a rare multisystem disorder. [neurology.org]
Also known as asymmetric polyneuropathy, in a non-diabetic this is suggestive of vasculitis. Palpable purpura. If patients have this in isolation, it is most likely due to cutaneous leukocytoclastic vasculitis. [en.wikipedia.org]
- Mononeuropathy
To elucidate the cause and mechanisms of nerve fiber lesions in a case of mononeuropathy multiplex (MNM). A 65-year-old man had a MNM for 6 months and no previous history of peripheral nerve impairment was known. [ncbi.nlm.nih.gov]
At least 4 criteria yields sensitivity and specificity of 85 and 99.7%. asthma (history of wheezeing or presently wheezing) eosinophilia > 10% on CBC mononeuropathy or polyneuropathy migratory or transient pulmonary opacities on chest x-ray (CXR) paranasal [en.wikipedia.org]
Symptoms and signs vary, depending on the organ or organ system predominantly affected: Peripheral nervous system: Patients usually present with asymmetric peripheral neuropathy, such as multiple mononeuropathy (mononeuritis multiplex) with signs of motor [msdmanuals.com]
Other features of WG include ocular inflammation, retroorbital masses, arthritis, multiple mononeuropathies, cerebral arteritis, and vasculitic skin lesions. WG has a variable course, depending on the sites of clinical involvement. [lupusinternational.com]
- Tingling
A 63-year-old man was admitted to the hospital because of difficulty in walking and a tingling sensation in his legs. On physical examination, edema of the bilateral legs was prominent. [neurology.org]
[…] other parts of the body Bluish color to the fingers and toes Signs of tissue death due to lack of oxygen such as pain, redness, and ulcers that do not heal Muscles and joints: Joint pain Leg pain Muscle weakness Brain and nervous system: Pain, numbness, tingling [medlineplus.gov]
Nerves in the arms and legs may result in painful tingling sensations, loss of feeling, and weakness. Gastrointestinal system. Patients may have significant abdominal pain, vomiting, and diarrhea. [medical-dictionary.thefreedictionary.com]
- Paresthesia
We report on a 69-year-old woman who presented with myalgia, hearing impairment, fever, night sweats, weight loss, muscular weakness, paresthesia, hypesthesia, and hypalgesia. [ncbi.nlm.nih.gov]
Other nervous system abnormalities include paresthesias, weakness, pain, and functional deficiencies such as foot drop. Mononeuritis multiplex is also typically found in the medium-vessel vasculitis, polyarterteritis nodosa (PAN). [clinicaladvisor.com]
Workup
Provides all the necessary tools to make a comprehensive diagnostic workup including data from ancillary techniques and molecular findings whenever appropriate. [books.google.es]
A skin biopsy is often part of the first-line investigations done in the workup of such patients. [ijdpdd.com]
The decision to continue her PTU while awaiting the diagnostic workup, rather than immediately discontinue the medication, was made due to her lack of other end-organ failure often associated with vasculitis. [westjem.com]
DAH in conjunction with other manifestations of systemic vasculitis (see section below on common findings) should trigger a workup to establish a diagnosis. [clinicaladvisor.com]
Treatment
Today, however, normal life spans are possible with proper treatment. The success of treatment is related to prompt diagnosis, aggressive treatment and careful follow-up to be sure that side effects from medications do not develop. [webmd.com]
Sorafenib is approved for advanced renal carcinoma treatment ( 8 ) and for advanced HCC ( 9 ). [jgo.amegroups.com]
Treatment with ganciclovir and anti-CMV immunoglobulin in addition to prednisolone medication for 6 months resulted in rapid improvement of the clinical symptoms without relapse. [ncbi.nlm.nih.gov]
Other areas of your body need to be close monitoring during treatment for SNV: nervous system heart lungs kidneys If any conditions affecting these areas develop, your doctor can prescribe appropriate treatment for those as well. [healthline.com]
Prognosis
ICU disease severity scores at admission were associated with mortality in the ICU but did not predict long-term outcome, unlike BVAS, which accurately predicted long-term outcome but not ICU prognosis for patients admitted to the ICU with active SNV. [ncbi.nlm.nih.gov]
Prognosis is better if disease remission is achieved within 18 months after diagnosis. Relapses are less common than in other vasculitic disorders. [msdmanuals.com]
What is the Prognosis of Necrotizing Vasculitis? (Outcomes/Resolutions) Outcomes for individuals with necrotizing vasculitis greatly vary depending on the location of inflammation and the severity of tissue damage. [dovemed.com]
Outlook (Prognosis) The outcome depends on the location of the vasculitis and the severity of tissue damage. [mountsinai.org]
Kawasaki disease: Initial treatment and prognosis. http://www.uptodate.com/contents/kawasaki-disease-initial-treatment-and-prognosis Zugriffsdatum: 20.4.2012 42 Suda K, Mastumoto M, Miyanishi S. [thieme-connect.com]
Etiology
Abstract Hepatitis A virus has been recognized as an etiologic factor of cutaneous necrotizing vasculitis in only 2 adult cases. [ncbi.nlm.nih.gov]
Due to the multiple etiologies associated with dermatologic emergencies, diagnosis may often be difficult. [westjem.com]
[…] vasculitides thought to represent hypersensitivity to an antigenic stimulus, such as a drug, infectious agent, or exogenous or endogenous protein; all disorders in this group involve the small vessels. nodular vasculitis a chronic vasculitis of unknown etiology [medical-dictionary.thefreedictionary.com]
Epidemiology
CONCLUSION: The new classification algorithm is a reliable method for classification of SNV for epidemiological purposes in our population. © 2013 Asia Pacific League of Associations for Rheumatology and Wiley Publishing Asia Pty Ltd. [ncbi.nlm.nih.gov]
Textbook of Systemic Vasculitis is an in-depth guide to the epidemiology, classification and genetics of systemic vasculitis as well as imaging, symptoms and management of the disease. [books.google.de]
These findings support a pro-aggregatory state that, in part, explains the thrombotic events shown by epidemiological studies. [journals.lww.com]
Kawasaki disease: Epidemiology and etiology. http://www.uptodate.com/contents/kawasaki-disease-epidemiology-and-etiology Zugriffsdatum: 20.4.2012 24 Grunebaum E, Blank M, Cohen S et al. [thieme-connect.com]
Pathophysiology
We also discuss the pathophysiology of nervous system involvement in patients with SNV. [ncbi.nlm.nih.gov]
Key words: Granulomatosis with polyangiitis, ANCA, Associated vasculitis, Cutaneous, LCV, Wegener’s granulomatosis Advertisement Journal of Molecular Pathophysiology SUBMIT YOUR ARTICLE NOW [scopemed.org]
The purpose of this case series is to describe the pathophysiology, diagnosis, and management of LINES. The authors’ institutional database was reviewed from 2008 to 2015, and they found 3 patients with LINES. [woundsresearch.com]
Prevention
In cases of tissue infection, antibiotics are used How can Necrotizing Vasculitis be Prevented? Since little is known about the cause of necrotizing vasculitis, there are no known methods of preventing the condition. [dovemed.com]
Emergency symptoms include: Changes in pupil size Loss of function of an arm, leg, or other body part Speech problems Swallowing difficulty Weakness Severe abdominal pain Prevention There is no known way to prevent this disorder. [mountsinai.org]
Prevention Because so little is known about what causes a particular individual to develop vasculitis, there are no known ways to prevent it. [medical-dictionary.thefreedictionary.com]
Emergency symptoms include: Changes in pupil size Loss of function of an arm, leg, or other body part Speech problems Swallowing difficulty Weakness Severe abdominal pain There is no known way to prevent this disorder. Habif TP. [medlineplus.gov]
Since it’s an autoimmune disorder with an unknown cause, there’s no known way to prevent the onset of SNV. This rare condition is treatable. However, damage to any area affected by SNV can’t be reversed. [healthline.com]