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Cutaneous Leukocytoclastic Vasculitis

Cutaneous small vessel vasculitis


Presentation

  • Since vasculitic paraneoplastic syndromes, including CLV, may develop before the clinical presentation of malignant tumors, the present case and others previously reported strongly suggest that in apparently idiopathic CLV, patients should be evaluated[ncbi.nlm.nih.gov]
  • An 85-year-old Japanese woman exhibited the rare presentation of multiple myeloma (immunoglobulin kappa chain type) with leukocytoclastic vasculitis and eosinophilia.[ncbi.nlm.nih.gov]
  • There were no signs of vasculitis present in the internal organs. After anti-tuberculosis treatment, his fever declined and the skin purpura were completely resolved.[ncbi.nlm.nih.gov]
  • By contrast, ACAs were present in only a minority of children with Henoch-Schönlein purpura and adults with IgA nephropathy and were not restricted to the IgA isotype.[ncbi.nlm.nih.gov]
  • The case history is presented of a patient who developed a cutaneous leukocytoclastic vasculitis after he had been treated with intravenous streptokinase because of an acute myocardial infarction.[ncbi.nlm.nih.gov]
Fever
  • After anti-tuberculosis treatment, his fever declined and the skin purpura were completely resolved. Although incidence is rare, tuberculosis should be considered as a possible cause of CLV.[ncbi.nlm.nih.gov]
  • A 71-year-old man presented with a high fever, polyarthralgia, petechiae and palpable purpura accompanied by livedoid change on his legs and feet.[ncbi.nlm.nih.gov]
  • He presented with a palpable purpura on both legs, low-grade fever, cough and expectoration, progressive dyspnea due to a massive left pleural effusion and a symmetric swelling on his ankles and wrists.[ncbi.nlm.nih.gov]
  • General symptoms such as fevers, weight loss and muscle aches may occur. Five to 25% of people with CSVV can have joint, genitourinary or gastrointestinal symptoms. This raises the possibility of internal involvement.[dermcoll.edu.au]
Weight Loss
  • The patient sought emergency treatment presenting with headache, arthralgia, cutaneous rash, and weight loss (4 kg) in the last 20 days.[ncbi.nlm.nih.gov]
  • General symptoms such as fevers, weight loss and muscle aches may occur. Five to 25% of people with CSVV can have joint, genitourinary or gastrointestinal symptoms. This raises the possibility of internal involvement.[dermcoll.edu.au]
  • The clinician should ask about the duration, onset, and presence any associated symptoms such as weight loss or fatigue (that would indicate a systemic cause). [10] It is important to distinguish between IgA and non-IgA vasculitis.[en.wikipedia.org]
  • She reported a 6-month history of dry cough, anorexia-cachexia, and 4 kg weight loss. On physical examination, she was seen to be asthenic, with a poor-to-middling general condition, body mass index 17.42, blood pressure 139/93, temperature 36.7 C.[archbronconeumol.org]
Malaise
  • Over the past 3 to 4 weeks, he has had malaise, nonproductive cough, and a decline in mental status but no fever, headache, nausea, light-headedness, hemoptysis, or melena.[consultant360.com]
  • There may also be fever, generalized discomfort (malaise), muscle or joint pain. Causes The exact cause of cutaneous necrotizing vasculitis is unknown.[rarediseases.org]
  • These forms present as a viral infection, with polyarthralgias, fever, malaise, fatigue and possible neurological manifestations.[clinicaldermatology.eu]
Chills
Gaucher Disease
  • We report the case of a Swedish man with an untreated N370S/L444P Gaucher disease who developed CLV at the age of 79 years.[ncbi.nlm.nih.gov]
Abdominal Pain
  • Abdominal pain occurred in 42 per cent of the male patients while none of the female patients had this symptom. Arthralgia occurred in 20 per cent of the patients.[ncbi.nlm.nih.gov]
  • Commonest symptom was abdominal pain (27.55%), followed by arthralgia (25.51%) and pruritus (24.49%). Majority of cases had unknown etiology (69.07%). Those with documented etiology included infection (25.51%) and drugs (5.10%).[nepjol.info]
  • For example, if the vasculitis is a manifestation of Henoch-Schönlein purpura, individuals may also experience abdominal pain or blood in the urine. [5] Cause [ edit ] Cutaneous vasculitis can have various causes including but not limited to medications[en.wikipedia.org]
  • The patient denied having a fever, abdominal pain, or arthralgia. Laboratory studies revealed normal platelet counts and coagulability. Renal function was unimpaired, and the results of urinalysis were insignificant.[e-ijd.org]
  • For example, a patient with purpura, nephritis, and abdominal pain caused by Henoch–Schönlein purpura usually has a good prognosis . . .[nejm.org]
Nausea
  • The most common adverse events associated with the quinolone class involve the gastrointestinal tract (nausea and diarrhea) and central nervous system (CNS) (headache and dizziness).[scienceopen.com]
  • Over the past 3 to 4 weeks, he has had malaise, nonproductive cough, and a decline in mental status but no fever, headache, nausea, light-headedness, hemoptysis, or melena.[consultant360.com]
  • A 34-year-old Hispanic man with a history of alcoholic cirrhosis and type 2 diabetes mellitus presented with 2 weeks of diarrhea, nausea, vomiting, abdominal pain, rash, and syncope.[amjmed.org]
Abdominal Cramps
  • Side effects were uncommon but, when they occurred, consisted mainly of abdominal cramping and/or diarrhea.[ncbi.nlm.nih.gov]
Arthritis
  • Autoimmune disorders such as systemic lupus erythematosus (SLE), dermatomyositis, and rheumatoid arthritis are characterised by circulating antibodies that target the individual's tissues.[dermnetnz.org]
  • Arthritis & Rheumatism. 65 (1): 1–11. doi : 10.1002/art.37715. PMID 23045170. 1950-, James, William D. (William Daniel) (2015-04-20). Andrews' diseases of the skin : clinical dermatology.[en.wikipedia.org]
  • The disorder is seen in rheumatoid arthritis and other diseases.[medical-dictionary.thefreedictionary.com]
  • Semin Arthritis Rheum 1998;27:319–324. Sigurgeirsson B, Lindelof B, Edhag O, Allander E: Risk of cancer in patients with dermatomyositis or polymyositis: A population-based study. N Engl J Med 1992;326:363–367.[karger.com]
Arthralgia
  • The patient sought emergency treatment presenting with headache, arthralgia, cutaneous rash, and weight loss (4 kg) in the last 20 days.[ncbi.nlm.nih.gov]
  • Arthralgia occurred in 20 per cent of the patients. The most common laboratory abnormalities were elevation of ESR, which was significantly more common in females than in males (P 0.047).[ncbi.nlm.nih.gov]
  • In all cases, systemic complications, such as renal failure, abdominal discomfort, and arthralgia, were not mentioned.[e-ijd.org]
  • Commonest symptom was abdominal pain (27.55%), followed by arthralgia (25.51%) and pruritus (24.49%). Majority of cases had unknown etiology (69.07%). Those with documented etiology included infection (25.51%) and drugs (5.10%).[nepjol.info]
  • Many patients develop skin lesions, particularly on the legs, accompanied by arthralgia and fever. The disorder is seen in rheumatoid arthritis and other diseases.[medical-dictionary.thefreedictionary.com]
Myalgia
  • Here, we report a 49-year-old male patient that presented with fever, myalgia, and multiple palpable purpura on both of his legs. The biopsy from the purpura yielded a histologic diagnosis of leukocytoclastic vasculitis.[ncbi.nlm.nih.gov]
  • The signs of systemic involvement include fever, myalgia (pain in the muscles), arthralgia (pain in the joints) hematuria (blood in the urine), melena (blood in the stools), abdominal pain, weakness, and numbness.[news-medical.net]
  • Besides this classical form, there is also a cutaneous polyarteritis nodosa that presents with systemic symptoms as well, including low-grade fever, myalgia, arthralgia and peripheral neuropathy, but especially with very painful erythematous nodules in[clinicaldermatology.eu]
  • Arthralgia or myalgia. Look at past medical history, including possible intravenous drug use, hepatitis, transfusion and travel.[patient.info]
Purpura
  • The absence of IgA ACAs in Henoch-Schönlein purpura argues against their being an epiphenomenon in vasculitis.[ncbi.nlm.nih.gov]
  • Purpura resolved after specific treatment of multiple myeloma and diagnosis of paraneoplastic purpura was asserted. We propose a brief review of the literature about skin involvement during multiple myeloma.[ncbi.nlm.nih.gov]
  • Here, we report a 49-year-old male patient that presented with fever, myalgia, and multiple palpable purpura on both of his legs. The biopsy from the purpura yielded a histologic diagnosis of leukocytoclastic vasculitis.[ncbi.nlm.nih.gov]
  • The patient was a 25-year-old man who had palpable purpura on his legs which showed leukocytoclastic vasculitis, and polyarteritis nodosa.[ncbi.nlm.nih.gov]
  • A 71-year-old man presented with a high fever, polyarthralgia, petechiae and palpable purpura accompanied by livedoid change on his legs and feet.[ncbi.nlm.nih.gov]
Palpable Purpura
  • Leukocytoclastic vasculitis (LCV), a disease characterized by inflammation of the small vessels, presents with palpable purpura, especially in the lower extremities.[ncbi.nlm.nih.gov]
  • The patient was a 25-year-old man who had palpable purpura on his legs which showed leukocytoclastic vasculitis, and polyarteritis nodosa.[ncbi.nlm.nih.gov]
  • He presented with a palpable purpura on both legs, low-grade fever, cough and expectoration, progressive dyspnea due to a massive left pleural effusion and a symmetric swelling on his ankles and wrists.[ncbi.nlm.nih.gov]
  • Here, we report a 49-year-old male patient that presented with fever, myalgia, and multiple palpable purpura on both of his legs. The biopsy from the purpura yielded a histologic diagnosis of leukocytoclastic vasculitis.[ncbi.nlm.nih.gov]
  • A patient with a cutaneous leukocytoclastic vasculitis manifest as palpable purpura had four lesions encircled on the day of presentation. Biopsies of these lesions were obtained sequentially at 0, 24, 48, and 120 hours.[ncbi.nlm.nih.gov]
Ulcer
  • Despite high-dose prednisone therapy, the pathergy test remained markedly positive; however, no new skin ulcerations appeared.[ncbi.nlm.nih.gov]
  • The morphologic type of skin lesions most commonly found were palpable purpura (51 patients), urticarial-like lesions (17 patients), ulcers (eight patients), erythematous plaques (five patients), and/or nodules (five patients).[ncbi.nlm.nih.gov]
  • الصفحة 55 - Stimulation of repair in chronic, nonhealing, cutaneous ulcers using platelet-derived wound healing formula. Surg Gynecol Obstet 1990;170:56-60. ‏[books.google.com]
  • These lesions can join together, become ulcerated, and may be associated with blood filled blisters. The rash of vasculitis is most common on the lower legs, areas of tight-fitting clothing and areas of trauma (Koebner phenomenon).[dermcoll.edu.au]
Urticaria
  • Since serologic testing has become available there have been several reports of cutaneous findings in association with hepatitis C virus infection, including vasculitis, cryoglobulinemia, urticaria, and lichen planus.[ncbi.nlm.nih.gov]
  • […] also: Cutaneous conditions, Category:Cutaneous conditions, and ICD 10… … Wikipedia Systemic vasculitis — Classification and external resources ICD 10 M30 M31 ICD 9 … Wikipedia urticarial vasculitis — a group of cutaneous vasculitides characterized by urticaria[medicine.academic.ru]
  • Cutaneous adverse reactions to anti-tuberculosis drugs have been reported in up to 5% of treated patients. 8 Common cutaneous reactions include pruritus, skin eruptions, maculopapular exanthems, urticaria, pustules, fixed reactions, and erythema nodosum[synapse.koreamed.org]
  • […] features of hypersensitivity vasculitis include: Prominent involvement of lower legs with fewer lesions on proximal sites Palpable purpura (purple, non- blanching papules and plaques ) Sometimes, petechiae and ecchymoses Non-purpuric erythematous or urticaria-like[dermnetnz.org]
  • Patients may present with skin symptoms such as lesions, including palpable purpura, petechiae, urticaria, ulcers, livedo reticularis, and nodules.[msdmanuals.com]
Petechiae
  • A 71-year-old man presented with a high fever, polyarthralgia, petechiae and palpable purpura accompanied by livedoid change on his legs and feet.[ncbi.nlm.nih.gov]
  • The most common presentation of CSVV is raised red-purple spots (purpura, 0.3 to 1 cm in diameter) and/or petechiae (purpuric lesions less than 3 mm in diameter) which do not disappear with pressure (i.e. non-blanching).[dermcoll.edu.au]
  • Patients may present with skin symptoms such as lesions, including palpable purpura, petechiae, urticaria, ulcers, livedo reticularis, and nodules.[msdmanuals.com]
  • The clinical features of hypersensitivity vasculitis include: Prominent involvement of lower legs with fewer lesions on proximal sites Palpable purpura (purple, non- blanching papules and plaques ) Sometimes, petechiae and ecchymoses Non-purpuric erythematous[dermnetnz.org]
  • On his abdomen, there were numerous scattered petechiae. Laboratory analysis showed nonketotic hyperglycemic decompensation.[pubs.sciepub.com]

Workup

  • IgA vasculitis is more likely to present with abdominal pain, bloody urine, and joint pain. [11] In the case that the cause is not obvious, a reasonable initial workup would include a complete blood count, urinalysis, basic metabolic panel, fecal occult[en.wikipedia.org]
  • A serological workup revealed a negative ANA, ANCA, Rheumatoid factor, Anti-cardiolipin antibodies, lupus anticoagulant, beta 2 glycoprotein, and Hepatitis C antibodies. Cryoglobulins and cold agglutinins were negative on two separate occasions.[ispub.com]
Cavitary Lesion
  • Imaging modalities revealed a thin-walled cavitary lesion in the right lung apex and scattered nodular opacities. Acid fast bacilli (AFB) were found in sputum and subsequently identified by culture as Mycobacterium avium-intracellulare (MAI).[ncbi.nlm.nih.gov]
Immunoglobulin G Decreased
  • Serum concentrations of immunoglobulin G decreased and plasma cells in bone marrow decreased.[ncbi.nlm.nih.gov]
Mycobacterium Chelonae
  • The patient experienced Bacille Calmette Guérin, Mycobacterium chelonae, and Salmonella enteritidis infection.[ncbi.nlm.nih.gov]
Hepatocellular Carcinoma
  • There are no other reports about erlotinib-induced leukocytoclastic vasculitis (LV) in the erlotinib-bevacizumab regimen for bone metastasis, from a relapsed hepatocellular carcinoma (HCC) in liver-transplanted patients.[ncbi.nlm.nih.gov]

Treatment

  • Response to treatment was judged according to the reduction in the number of lesions.[ncbi.nlm.nih.gov]
  • Skin lesion improved after cessation of the two drugs and treatment with corticosteroids.[ncbi.nlm.nih.gov]
  • Due to its more chronic nature and cumulative toxicities that patients develop from multiple lines of treatments, a number of symptoms are associated with multiple myeloma.[ncbi.nlm.nih.gov]
  • Careful examination of ocular inflammation is important in monitoring systemic disease and treatment.[ncbi.nlm.nih.gov]
  • This rash completely resolved with withdrawal of gabapentin and steroidal treatment. This cutaneous adverse reaction to gabapentin has not been reported in the medical literature.[ncbi.nlm.nih.gov]

Prognosis

  • We suggest that absence of autoimmune phenomena and immune-complex in the skin lesions of CLV may be a hallmark of good prognosis.[ncbi.nlm.nih.gov]
  • Vincent's Hospital Melbourne to determine the classification, aetiology, severity and prognosis of this population of patients.[ncbi.nlm.nih.gov]
  • An analysis of the group was made to determine the relationship of cutaneous to systemic disease, the incidence and importance of laboratory abnormalities, and the prognosis of patients with cutaneous LV and to compare these results with previously published[ncbi.nlm.nih.gov]
  • Multiple myeloma is a very heterogeneous disease comprising a number of genetic entities that differ from each other in their evolution, mode of presentation, response to therapy, and prognosis.[ncbi.nlm.nih.gov]
  • When IgG or IgM immune complexes are involved, the prognosis is better, since there is a less likelihood of systemic pathologies. Management and Prognostic Factors In general, individuals with hypersensitivity vasculitis have a very good prognosis.[news-medical.net]

Etiology

  • Abstract Forty-five patients with histologically proved cutaneous leukocytoclastic vasculitis were studied with regard to the clinical features, laboratory findings and etiology.[ncbi.nlm.nih.gov]
  • The syndrome may be associated with multiple etiologic agents or diseases. Malignancy, usually lymphoreticular, has been present in rare patients (less than 1%).[ncbi.nlm.nih.gov]
  • Its etiology is known to include drugs, infection, collagen tissue disease, and malignancy, but LCV caused by anti-tuberculosis drugs is very rarely seen.[ncbi.nlm.nih.gov]
  • As such, the patient might go undiagnosed for years while having symptoms of LCV or iritis of unknown etiology. This article discusses the correlation seen with LCV and iritis.[ncbi.nlm.nih.gov]
  • Majority of cases had unknown etiology (69.07%). Those with documented etiology included infection (25.51%) and drugs (5.10%). Leukocytosis was commonest laboratory abnormality.[nepjol.info]

Epidemiology

  • Objectives: To study epidemiology, etiology, clinical and laboratory features and treatment outcomes in LCV patients.[nepjol.info]
  • […] how these impact on activity and toxicity; (2) resistance mechanisms (mutations in target genes, efflux pumps); (3) their pharmacodynamic properties (AUC/MIC and Cmax/MIC ratios; mutant prevention concentration and mutant selection window); and (4) epidemiological[scienceopen.com]
  • Epidemiology The disease is in many ways similar to Henoch-Schönlein purpura.[patient.info]
  • Glucocorticoids Although cutaneous small-vessel vasculitis is not rare, few epidemiological studies and no large controlled therapeutic trials have been conducted.[rheumatologyadvisor.com]
  • The proper diagnosis and management of leukocytoclastic vasculitis remains a challenge for many clinicians and this report serves to give a brief overview of this condition, its causes, epidemiology, diagnosis and management.[ispub.com]
Sex distribution
Age distribution

Pathophysiology

  • The exact pathophysiological mechanisms of ANCA and adhesion molecules are unclear. If IgA immune complexes are the dominant immunoglobulins involved, there is a greater chance of systemic involvement in both children and adults.[news-medical.net]
  • This finding on histological examination is termed “leukocytoclastic vasculitis”. [5] Considering the wide range of potential causes leading to cutaneous small vessel vasculitis, there are subtle variations in the underlying pathophysiology for each cause[en.wikipedia.org]
  • Pharmacotherapy: a pathophysiological approach, 4th ed. Norwalk, Appleton & Lange 1999: 1510. 2002 Lippincott Williams & Wilkins, Inc.[journals.lww.com]

Prevention

  • Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets[books.google.com]
  • How can cutaneous small vessel vasculitis be prevented? Flares of cutaneous small vessel vasculitis can be minimised by rest, compression and elevation of lower legs.[dermnetnz.org]
  • […] based on an understanding of: (1) their chemical structures and how these impact on activity and toxicity; (2) resistance mechanisms (mutations in target genes, efflux pumps); (3) their pharmacodynamic properties (AUC/MIC and Cmax/MIC ratios; mutant prevention[scienceopen.com]
  • A worldwide, phase III, randomized, controlled, safety and efficacy study of a sirolimus/cyclosporine regimen for prevention of acute rejection in recipients of primary mismatched renal allografts. Transplantation 2001; 71: 271. 3.[journals.lww.com]
  • Experimental procedures that have been helpful in treating some cases of vasculitis include: plasmapheresis intravenous gamma globulin cyclosporine (cyclosporin A, Neoral , Sandimmune ) or tacrolimus (Protopic ), medications used to prevent organ rejection[resources.lupus.org]

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