1/19. Dermatological adverse effects with the antimalarial drug mefloquine: a review of 74 published case reports.mefloquine is a relatively new antimalarial drug which has been associated with a wide variety of adverse effects, including skin reactions. In order to evaluate the range and frequency of mefloquine's dermatological effects, we searched the scientific literature for published case reports of such effects. We found 74 case reports, published between the years 1983 and 1997. pruritus and maculopapular rash are the dermatological effects most commonly associated with mefloquine: their approximate frequency is 4-10% for pruritus, and up to 30% for nonspecific maculopapular rash. Adverse effects associated less commonly with mefloquine include urticaria, facial lesions and cutaneous vasculitis. One case of stevens-johnson syndrome and one fatal case of toxic epidermal necrolysis occurred. Appropriate primary studies of mefloquine use should be carried out to elucidate the epidemiology and aetiology of dermatological and other adverse effects of the drug.- - - - - - - - - - ranking = 1keywords = vasculitis (Clic here for more details about this article) |
2/19. Infantile Henoch-Schonlein purpura.Henoch-Schonlein purpura is a common cause of vasculitis in children. This condition is unusual in infants and children younger than 2 years. We describe a 4-month-old infant with infantile Henoch-Schonlein purpura and review the clinical spectrum, differential diagnoses, and the histopathologic features of the disease. Its relations to Henoch-Schonlein purpura in older children are discussed.- - - - - - - - - - ranking = 1keywords = vasculitis (Clic here for more details about this article) |
3/19. A woman with leg cramps and rash.A 68-year-old woman presented with generalized weakness and a rash on her lower extremities. The weakness began one week earlier, shortly after treatment for leg cramps and two days before the rash appeared. She had also had blood-streaked sputum a few days before admission. There was no history of bleeding diathesis, nose bleeding, hematuria, vasculitis, muscle or joint aches, cinchonism, or exposure to tuberculosis. She denied fever, chills, night sweats, leg pain or swelling, chest pain, or abdominal pain.- - - - - - - - - - ranking = 1keywords = vasculitis (Clic here for more details about this article) |
4/19. The schnitzler syndrome. Four new cases and review of the literature.The schnitzler syndrome is characterized by a chronic urticarial eruption with a monoclonal IgM gammopathy. The other signs of the syndrome include intermittent elevated fever, joint and/or bone pain with radiologic evidence of osteosclerosis, palpable lymph nodes, enlarged liver and/or spleen, elevated erythrocyte sedimentation rate, and leukocytosis. The mean delay to diagnosis is more than 5 years, and this syndrome is of concern to internists and many medical specialists. patients with this syndrome are often initially considered to have lymphoma or adult-onset Still disease, which are the main differential diagnoses. However, hypocomplementic urticarial vasculitis, systemic lupus erythematosus, cryoglobulinemia, acquired C1 inhibitor deficiency, hyper IgD syndrome, chronic infantile neurologic cutaneous and articular (CINCA) syndrome, and Muckle-Wells syndrome should also be excluded, because diagnosis relies on a combination of clinical and biologic signs and there is no specific marker of the disease. The disease pursues a chronic course, and no remissions have yet been reported. Disabling skin rash, fever, and musculoskeletal involvement are the most frequent complications. Severe anemia of chronic disease is another serious complication. The most harmful complication, however, is evolution to an authentic lymphoplasmacytic malignancy, which occurs in at least 15% of patients. This hematologic transformation can occur more than 20 years after the first signs of the disease, thus patients deserve long-term follow-up. Treatment is symptomatic and unsatisfactory. The skin rash is unresponsive to treatment, and nonsteroidal antiinflammatory drugs, antihistamines, dapsone, colchicine, and psoralens and ultraviolet A (PUVA) therapy give inconstant results. fever, arthralgia, and bone pain often respond to nonsteroidal antiinflammatory drugs. In some patients, these symptoms and/or the presence of severe inflammatory anemia require steroids and/or immunosuppressive treatment, which ameliorate inflammatory symptoms but do not change the course of the skin rash.- - - - - - - - - - ranking = 1keywords = vasculitis (Clic here for more details about this article) |
5/19. Benign cutaneous Degos' disease: a case report with emphasis on histopathology as papules chronologically evolve.The following case report details a 53-year-old man with a 6-year history of the benign cutaneous or skin-limited form of Degos' disease. Clinically, the patient demonstrated a diffuse eruption of papules on the upper trunk and arms. Many papules demonstrated the classic porcelain-white centers characteristic of Degos' disease, but others exhibited different clinical morphologies that corresponded to the evolutionary stages of papules originally described by Degos. Over the course of several clinic visits, the patient underwent a total of 5 punch biopsies, the histologies of which were correlated with their clinical morphologies. Early papules were skin-colored and demonstrated a superficial and deep perivascular, periadnexal, and perineural chronic inflammatory cell infiltrate associated with interstitial mucin deposition. The overlying epidermis showed a mild vacuolar interface reaction and the histologic appearances at this early stage resembled tumid lupus erythematosus. Fully developed papules were raised with umbilicated porcelain-white centers and a surrounding erythematous rim. Histologically these exhibited a prominent interface reaction with squamatization of the dermo-epidermal junction, melanin incontinence, epidermal atrophy, and a developing zone of papillary dermal sclerosis that resembled the early stages of lichen sclerosus et atrophicus in miniature. These interface reactions were invariably confined to the central portion of the punch biopsy specimen, corresponding to the central porcelain-white area seen clinically. Additional features of fully developed papules included a prominent lymphocytic vasculitis affecting venules, a mild periadnexal infiltrate of neutrophils and/or eosinophils, and interstitial mucin deposition. In late-stage papules, the porcelain-white areas were better developed and the lesion flattened. Histologically, the degree of inflammation was generally sparse and the overall picture mirrored the classic histologic description of Degos' disease with a central roughly wedge-shaped zone of sclerosis surmounted by an atrophic epidermis and hyperkeratotic compact stratum corneum. These late-stage papules closely resembled a miniaturized version of fully developed lichen sclerosus et atrophicus confined to the center of the punch biopsy specimen.- - - - - - - - - - ranking = 1keywords = vasculitis (Clic here for more details about this article) |
6/19. Chronic infantile neurological cutaneous and articular syndrome: two new cases with rare manifestations.CINCA/IOMID is a systemic inflammatory disorder of unknown aetiology that resembles congenital infection and systemic juvenile chronic arthritis (JCA). This disorder is characterized by neonatal onset, persistent rash, ocular inflammatory lesions, and progressive articular and neurological involvement. We report two new patients with this syndrome. Both children presented periodic bouts of cutaneous rash, fever, organomegaly, articular involvement with typical radiological features, and developmental delay. One of the patients presented neonatal jaundice and elevation of liver enzymes; inflammatory infiltrates were observed in the liver biopsy. The other patient showed retinal vasculitis detected at age 18 mo on fundoscopy and fluorescent angiography. Therapy with azathioprine was associated with prolonged remission of this complication. In both cases, the disease was diagnosed after some delay. Conclusion: Early hepatitis and retinal vasculitis are rare features of CINCA/IOMID that may help differentiate this syndrome from JRA. azathioprine may have induced the remission of vasculitis in one case.- - - - - - - - - - ranking = 3keywords = vasculitis (Clic here for more details about this article) |
7/19. Atypical presentation of churg-strauss syndrome: another "forme fruste" of the disease?Vasculitis is a clinicopathologic process characterized by inflammation and damage to blood vessels. A broad and heterogenous group of syndromes may result from this process, because any type, size, and location of blood vessel may be involved. The cause of these conditions remains unclear, but an autoimmune inflammatory process, characterized by involvement of both neutrophils and endothelial cells, seems to play an important role. In 1951, Churg and Strauss described a clinical syndrome of severe asthma, hypereosinophilia with eosinophilic infiltrates, eosinophilic vasculitis, and granulomata in various organs. asthma may precede this vasculitis by many years. We report a case of anti-neutrophil cytoplasmic antibody-positive, pauci-immune, crescentic, necrotizing glomerulonephritis with peripheral and interstitial eosinophilia but without asthma. This is very unusual in churg-strauss syndrome.- - - - - - - - - - ranking = 2keywords = vasculitis (Clic here for more details about this article) |
8/19. Successful treatment of mesenteric vasculitis caused by Henoch-Schonlein purpura with methylprednisolone pulse therapy.Although mesenteric vasculitis due to Henoch-Schonlein purpura (HSP) is relatively uncommon, it is the most life-threatening manifestation associated with high mortality. We describe a 15-year-old boy with HSP who had massive gastrointestinal bleeding and ileus but delayed onset of the purpuric rash. Abdominal ultrasonography revealed thickening of both small and large intestinal walls, and CT found prominent mesenteric vessels with comb sign and double wall of the bowel. These findings were consistent with mesenteric vasculitis and bowel ischaemia. The ischaemic intestine recovered after methylprednisolone pulse therapy and surgical intervention was avoided. Our report suggests that corticosteroid pulse therapy may help controlling HSP with massive gastrointestinal haemorrhage and ischaemic bowel due to widespread mesenteric vasculitis.- - - - - - - - - - ranking = 7keywords = vasculitis (Clic here for more details about this article) |
9/19. acute generalized exanthematous pustulosis due to sulfamethoxazol with positive lymphocyte transformation test (LTT).We studied an acute generalized exanthematous pustulosis (AGEP) due to sulfamethoxazol in a 48-year-old woman with unusual findings in allergy testing. The histological picture provided evidence for a pustular drug eruption and leukocytoclastic vasculitis. skin testing with sulfamethoxazol was negative for immediate-type reaction (scratch test) and delayed-type reaction (epicutaneous testing). A lymphocyte transformation test (LTT) showed a significant lymphocyte stimulation (stimulation index 5.04/2.61) toward sulfamethoxazol (200/100 mg/ml) by measuring the rate of built-in tritium-thymidine in the DNS of the patients lymphocytes, implicating a drug-specific hypersensibility of lymphocytes; we could be dealing with a combined type III and IV reaction by Coombs and Gell in this case. LTT may play a possible role in the determination of drug allergy in AGEP despite negative skin testing.- - - - - - - - - - ranking = 4.1987700461116keywords = leukocytoclastic, leukocytoclastic vasculitis, vasculitis (Clic here for more details about this article) |
10/19. A case of acute hemorrhagic edema of infancy.Acute hemorrhagic edema of infancy is an unusual form of leukocytoclastic vasculitis occuring in children from the age 4 months to 2 years. The etiology remains unknown. Numerous studies, however, suggest acute hemorrhagic edema of infancy as an immune-mediated vasculitis in response to a variety of antigenic stimuli. We report a case of an acute hemorrhagic edema of infancy; 11-month-old boy with a history of fever for 3 days and a history of purpuric rash on the extremities, trunk, buttock and oral mucosa for 2 days.- - - - - - - - - - ranking = 5.1987700461116keywords = leukocytoclastic, leukocytoclastic vasculitis, vasculitis (Clic here for more details about this article) |
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