Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A retrospective study over an eight-year period of 33 episodes of leg ulceration in 26 patients with rheumatoid arthritis requiring inpatient management is reported. the aetiology of the ulcers was found to be multifactorial. The most common factors were venous insufficiency (45.5%), trauma or pressure (45.5%) and arterial insufficiency (36.4%). Vasculitis (18.2%) and Felty's syndrome (12.1%) were less frequent causes, and pyoderma gangrenosum was rare. Most patients had seropositive erosive disease with high rheumatoid factor titres and significant functional impairment; over half were on maintenance corticosteroids. Colonisation of the ulcers by organisms, predominantly Staphylococcus aureus, was common (69.7%). Skin grafting was required in 63.3%, but the rate of complete take was only 42.9% despite multiple attempts. Hospitalisation was prolonged (mean 47.9 days) and the recurrence rate requiring further hospitalisation was 26.9%. The diagnosis of vasculitis and the limited role of biopsy in establishing its presence are discussed.
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PMID:Leg ulcers in rheumatoid arthritis. 223 32

We report 12 cases of leucocytoclastic vasculities associated with myelocytic (7 cases) or lymphocytic (5 cases) blood diseases. The clinical features, laboratory abnormalities and pathological findings are presented. In all cases a drug-induced and/or infective origin could be ruled out, and patients with cryoglobulinaemia were excluded. As in other cases found in the literature, in some patients vasculitis was present several weeks or months before the blood disease was discovered, while in others both conditions developed simultaneously or vasculitis appeared in the course of the blood disease. None of these three possibilities seemed to make the prognosis worse. The pathogenesis of the vasculitis-malignant blood disease association is uncertain. Immune complexes of tumoral origin undoubtedly have some responsibility, but some deficiency of phagocytic cells or chemotactic factors also play a role. Cases such as ours enable the significance of vasculitis to be understood when it occurs in a patient with malignant blood disease, but above all they should prompt investigations for malignant blood disease in patients with cutaneous and/or systemic vasculitis, as is being done in patients with pyoderma gangrenosum or Sweet's syndrome.
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PMID:[Leukocytoclastic vasculitis and malignant hematologic diseases. (12 cases)]. 248 1

Three cases and a pathogenic discussion. We report three cases of clinically typical pyoderma gangrenosum (PG) remarkable for the presence, at histology, of inflammatory vascular lesions of varying intensity. Case No. 1. A 15-year old boy without previous pathology was admitted for necrotic cutaneous lesions typical of PG on both legs. Biopsy in the peri-ulcerous rim showed cellular infiltration of the dermis, principally by granulocytes, associated with granulocytic vasculitis with fibrinoid necrosis (fig. 1 and 2). Laboratory examinations only showed a decrease of CH 50, and attempts at making an aetiological diagnosis of this case of PG met with failure. PG regressed under treatment with disulone combined with topical corticosteroid therapy. Case No. 2. A 62-year old woman with no previous severe disease was admitted for PG of her right leg at the ulcero-necrotic stage (fig. 3). Biopsy in the PG rim showed infiltration of the dermis, predominantly by granulocytes, associated with granulocytic vasculitis and fibrinoid necrosis of the walls of small vessels (fig. 4). Investigations in search of a cause revealed benign thyroid gland hyperplasia. PG was cured after a 5-week oral treatment with minocycline. Case No. 3. This 63-year old woman with arterial hypertension was admitted for PG of both legs. Some lesions at a very early stage were bullous (fig. 5). Biopsy in the peripheral swelling showed polymorphous cellular infiltration of the dermis with a predominance of granulocytes. These cells infiltrated the vessels the walls of which were turgid but without fibrinoid necrosis (fig. 6).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Pyoderma gangrenosum and vasculitis. Pathogenic discussion apropos of 3 cases]. 296 77

A new case of Sweet's syndrome (acute febrile neutrophilic dermatosis) associated with a malignant hemopathy is presented. The blood disease was a chronic myelomonocytic dysmyelopoiesis which was discovered during the eruption and resulted in the patient's death within a few months, probably through acutization. The skin lesions were atypical, bullous and ulcerated. On this occasion, the international literature concerning all cases of Sweet's syndrome associated with malignant or premalignant hemopathies is reviewed. Several concepts emerge from this study: the association is frequent (about 20 p. 100 of all published cases of Sweet's syndrome); there is a strong predominance of granulocytic hemopathies over lymphoplasmocytic and monocytic hemopathies; the blood disease is revealed by the skin eruption in some 50 p. 100 of the patients; there are frequent chronological relations between Sweet's syndrome and the events that occur in the course of the hemopathy; finally, the association is usually of poor prognosis. A comparison with Sweet's syndrome unassociated with a blood disease showed only three significant points: the frequency of bullous lesions, of the initial anaemia (the most important element) and of extreme figures in leucocyte counts (leucopenia or major hyperleukocytosis). The atypical character of the skin lesions in the patient presented here incites to discuss the nosological relationship between Sweet's syndrome and bullous pyoderma, an entity closely associated with hemopathies. It has recently been suggested by several authors that this anatomico-clinical kinship should be turned into a wide spectrum of acute neutrophilic dermatoses, with typical Sweet's syndrome at one end and Pyoderma gangrenosum at the other end. The interface between this spectrum and haemopathies seems to be maximum at its intermediate stage: the bullous and superficially ulcerated lesions. The aetiology and pathogenesis of this new nosological entity are uncertain. The presence of chemoattractants or of polymorphonuclear cell abnormalities is still open to discussion. The relationship between the entity and leukocytoclastic vasculitis has recently been questioned.
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PMID:[Acute febrile neutrophilic dermatosis and malignant hematologic diseases: report of a new bullous case and review of the literature]. 305 44

Two patients with the lupus anticoagulant exhibited unusual cutaneous manifestations. They both fulfilled four criteria for systemic lupus erythematosus and had experienced deep venous thrombosis. The first patient suffered from a leg ulcer that resembled a pyoderma gangrenosum. The second patient presented erythematous and purplish macules on the fingertips. The histologic studies showed only microthrombosis in the dermal vessels without vasculitis, although such lesions in systemic lupus erythematosus are usually attributed to vasculitis. The association of these cutaneous lesions with lupus anticoagulant has never been reported. It is likely that this association is not fortuitous. After a review of the literature, it seems possible to individualize a new syndrome characterized by the presence of a subgroup of antiphospholipid antibodies. Thrombosis, spontaneous abortions, neurologic manifestations, pulmonary hypertension, positive results of a Coombs' test, and thrombocytopenia can be included in this syndrome, which overlaps with systemic lupus erythematosus. Certain cutaneous symptoms are associated with the presence of lupus anticoagulant or other antiphospholipid antibodies: leg ulcers, distal cutaneous ischemia, widespread cutaneous necrosis, and livedo. They can be considered as the dermatologic manifestations of this syndrome.
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PMID:Cutaneous manifestations associated with the presence of the lupus anticoagulant. A report of two cases and a review of the literature. 309 56

Out of 210 cases of ulcerative colitis 41 had skin and mucosal lesions: skin lesions in 23 (11%), oral ones in 11 (5.2%) and perianal in 16 (7.6%). Cutaneous lesions were: maculopapular rashes (5.2%), erythema nodosum (2.9%), pyoderma gangrenosum (1.4%), papulopustular lesions, vasculitis and erythema multiforme (1% each). Aphthae were the commonest oral complication (4.3%) and fissures and fistula the more prevalent perianal ones (4.3% and 3.4%). More than one mucocutaneous complication occurred in 17.1% of the patients and in 18 patients there were associated articular (36.6%), ocular (9.8%) and hepatic (2.4%) changes. The colitic type of arthritis and keratoconjunctivitis sicca were the commoner joint and eye manifestations.
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PMID:[Cutaneous manifestations of ulcerative colitis]. 331 61

Sixty-three patients with pyoderma gangrenosum were seen and studied at the Mayo Clinic from 1971 to 1980. Biopsies from the erythematous border or necrotic edge of the pyoderma gangrenosum lesions usually demonstrated a characteristic pathogenic morphologic evolution. The early lesions revealed mild to moderate perivascular lymphocytic infiltrate associated with endothelial swelling. The fully developed lesions demonstrated necrosis in addition to a dense lymphocytic infiltration surrounding as well as involving the blood vessels. Extravasation of erythrocytes and thrombosis sometimes were seen. Ulceration, infarction, and abscess formation were found in the later stages of evolution. Direct immunofluorescence results were positive in the blood vessels of 36 of 65 (55%) specimens. IgM, C3, and fibrin were found in the papillary and reticular dermal vessels. IgG and IgA were only occasionally present. Pyoderma gangrenosum appears to be a reactive process that is manifested as a vasculitis. Biopsy material from the advancing active erythematous border has early characteristic dermatopathologic findings of lymphocytic vasculitis. Cutaneous vascular immune deposits suggest an immune pathogenesis of either an immune complex disease or lymphocytotoxic reaction.
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PMID:Histopathologic and immunopathologic study of pyoderma gangrenosum. 353 32

Four patients with pyoderma gangrenosum were treated with hyperbaric oxygen to prepare the wounds for skin grafting. Each wound responded to a course of daily hyperbaric oxygen with reduction of infection and increased capillary angiogenesis. During follow-up periods of 12 to 30 months, all wounds remained healed. Although the exact etiology of pyoderma gangrenosum is unknown, vasculitis with wound ischemia and infection are prominent components. Inspired oxygen partial pressures of 1100 to 1300 mmHg elevate wound oxygen tension despite relative ischemia. The impaired intracellular bacterial killing of hypoxic leukocytes is corrected during each day's 2-hour bolus of hyperbaric oxygen. Daily wound oxygenation increases collagen production by fibroblasts to support capillary angiogenesis.
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PMID:Pyoderma gangrenosum: skin grafting after preparation with hyperbaric oxygen. 354 80

The authors describe three cases of refractory anaemia with an excess of myeloblasts in the bone marrow (RAEM), associated with pyoderma gangrenosum (PG) and vasculitis. The first patient was an 85-year old man whose RAEM had begun in 1979. In 1985, he developed pyoderma gangrenosum in the popliteal fossa and on the right heel. Histology confirmed the diagnosis. A direct immunofluorescence test on the biopsy specimen was negative. Apart from the haematological syndrome, there were no laboratory abnormalities. The cutaneous lesions disappeared after 7 weeks of treatment with prednisolone 20 mg per day. The blood disease remained unchanged. The second patient was a 71-year old woman with RAEM since 1982. In 1984, she presented with lesions of cutaneous vasculitis located on the anterior aspect of the upper third of her left leg. There was neither arthralgia nor fever, and no history of drug toxicity or infection. Beside RAEM, polyclonal hyperglobulinaemia was present. Histological examination of the skin showed evidence of vasculitis with fibrinoid necrosis of vascular walls and perivascular lympho-histiocytic infiltrate with granulocytes and slight leucocytoclasia. Direct immunofluorescence testing of the skin demonstrated intravascular complement deposits. There were no circulating immune complexes; measurements of complement and complement fractions gave normal values; no cryoglobulin was found. The cutaneous lesions recurred on two occasions in 6 months, although no drug toxicity or infection could be elicited and the haematological syndrome was unaffected. The third patient was a 67-year old man with RAEM since 1982. In 1983, an ulcero-necrotic lesion spontaneously developed on his right leg.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Skin manifestations of refractory anemia with excess of blasts (RAEB)]. 357 13

The clinical, histologic, and immunofluorescent findings in 86 cases of pyoderma gangrenosum seen at the Mayo Clinic between 1970 and 1983 were reviewed. Males and females were affected in equal numbers. The most frequent site of lesions was the leg. Sixty-seven patients (78 per cent) had associated systemic disease, with arthritis and inflammatory bowel disease being commonest. Cutaneous histopathologic changes varied with the site of biopsy. Lymphocytic vasculitis was predominant in the zone of erythema peripheral to the area of ulceration, while neutrophilic infiltrate and abscess formation were more prominent centrally. In most cases studied, direct immunofluorescence showed immunoglobulins and complement deposited in and around superficial and deep dermal vessels.
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PMID:Pyoderma gangrenosum: a review of 86 patients. 388 78


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