Cases reported "Skin Ulcer"

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1/14. Perianal cytomegalovirus ulcer in an hiv infected patient: case report and review of literature.

    We report the case of a 25-year-old man with acquired immunodeficiency syndrome, presenting with perianal ulcer and diarrhea. He had positive immunocytochemical tests for cytomegalovirus (CMV) in circulating polymorphonuclear cells (PMN). The biopsy specimen was suggestive of CMV infection, and specific immunoperoxidase for CMV antigens positively stained endothelial cells and fibroblasts. In this report we review cutaneous CMV infection in immunocompromised patients.
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2/14. Detection of cytomegalovirus antigens in phagocytosed serum complexes from a patient with rheumatoid arthritis, vasculitis, peripheral neuropathy, cutaneous ulceration, and digital gangrene.

    A patient with rheumatoid arthritis, vasculitis, peripheral neuropathy, cutaneous ulceration, and digital gangrene was studied. Circulating immune complexes were detected by C1q binding although serum complement levels were within the normal range. Immunofluorescent staining of buffy coat cells with specific antisera showed the presence of IgG and IgM in phagocytosed inclusions but complement c3 was not detected. A monoclonal antibody specific for cytomegalovirus detected antigens in phagocytosed inclusions on one occasion. These results may suggest that cytomegalovirus antigens are a hitherto unidentified component of serum complexes in patients with rheumatoid arthritis and may contribute to the pathogenesis of the vasculitic complications of rheumatoid arthritis by participating in immune complex formation.
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3/14. A case of cytomegalovirus colitis following immunosuppressive treatment for pyoderma gangrenosum.

    We report a case of pyoderma gangrenosum (PG) complicated by cytomegalovirus (CMV)-induced colitis. A 79-year-old woman with PG was treated with corticosteroid and cyclosporin. She had blood in her stool and advancing anemia during the treatment. A colonoscopic biopsy specimen from the colon revealed typical CMV-infected cells with CMV inclusions confirmed by immunohistochemistry. Furthermore, there were many CMV-antigen-positive leukocytes, suggesting an active CMV infection, which is serious in compromised hosts. Although ulcerative colitis and Crohn's disease are well known as complications of PG, CMV enterocolitis should be considered in the differential diagnosis of enterocolitis in immunocompromised patients.
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4/14. Unusual thromboses associated with protein s deficiency in patients with acquired immunodeficiency syndrome: case reports and review of the literature.

    Recent reports indicate that patients infected with hiv are at increased risk for the development of thrombosis. Among other possibilities, an acquired deficiency of protein S (PS), one of the plasma's natural anticoagulants, might explain this tendency. PS deficiency can be classified in three types depending on the levels of total and free protein (antigenic assays) as well as anticoagulant activity (functional assay). Although the prevalence of inherited PS deficiency is not known because of its rarity, several conditions can lead to acquired forms of the disease. We report two AIDS patients with coexistent type III PS deficiency and thrombosis. Our first patient presented with bilateral chronic leg ulcers and a skin biopsy revealed dermal microthromboses. On laboratory evaluation he had PS deficiency and was started on anticoagulation, but was lost to follow-up. The second patient presented with hepatic vein thrombosis (budd-chiari syndrome) and was also PS deficient. On long-term anticoagulation, she experienced resolution of the thrombosis. Neither patient had prior personal or family history of venous thrombosis, nor acquired risk factors such as immobility, acute infection, recent surgery, or hormonal therapy. The literature contains a few reports of skin ulcers and budd-chiari syndrome associated with PS deficiency, although none in AIDS patients. While a larger number of studies describe an association between PS deficiency and hiv infection, the causal effect of this deficiency on the thrombophilic tendency in AIDS has not been established. We propose that awareness of the increased risk for thrombosis in hiv infection is important to the understanding of disease pathophysiology and management of these patients.
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5/14. Evaluation of clinically normal appearing skin for systemic infectious diseases in human immunodeficiency virus seropositive patients.

    The observation of a human immunodeficiency virus (hiv)-infected patient with cutaneous ulcers which contained both fungal and viral pathogens, and in which the fungal organisms were grown in culture yet could not be demonstrated microscopically, suggested the possibilities: 1) that the skin lesions might have been primarily caused by the viral pathogen, and 2) that the concurrent presence of that fungal pathogen was coincidental and perhaps secondary to fungemia. Assuming that these postulates are valid, it is reasonable to hypothesize that fungal organisms would have been detected if perilesional or distal, clinically normal appearing, skin had been evaluated. This hypothesis could be further assessed by performing a prospective study of clinically normal appearing skin for pathogens of suspected systemic infectious diseases in hiv seropositive patients. Since the quantity of infectious pathogens in nonlesional skin may be limited, it might be difficult to grow the organisms in culture or demonstrate them microscopically using standard methods. Therefore, in addition to routine cultures and histologic evaluation, immunohistochemical techniques using monoclonal antibodies to pathogen antigens and dna amplification techniques based upon the polymerase chain reaction should be used to enhance the detection of the infectious pathogens.
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6/14. mouth and genital ulcers with inflamed cartilage: magic syndrome. Five patients with features of relapsing polychondritis and Behcet's disease.

    Five patients with features of coexistent relapsing polychondritis and Behcet's disease are described. review of the literature supports the overlap of the clinical manifestations of these two conditions. A common immunologic abnormality is likely, and elastin is cited as a possible target antigen. The "mouth and genital ulcers with inflamed cartilage (magic) syndrome" is the proposed name for this entity.
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7/14. behcet syndrome: with immunologic evaluation.

    A case of behcet syndrome with immunologic evaluation, including screening of a vulvar ulcer for IgG, IgM, IgA, and fibrinogen by direct fluorescent microscopy is presented. Attempts were made to demonstrate cellular and humoral immune responses to mucosal antigens by lymphoblast transformation in the presence of cadaver esophageal mucosal extracts and indirect immunofluorescence using autologous serum and mucosal tissue. Serial measurements of percentages of total T, active T, and B lymphocyte populations, and lymphocyte response to phytohemagglutinin (PHA) stimulation during the course of behcet syndrome are also presented. Clinical evaluation, histology of a Behcet vulvar ulcer, and a 2-year followup with good response to chlorambucil are reviewed.
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8/14. Familial defect of polymorph neutrophil phagocytosis associated with absence of a surface glycoprotein antigen (OKMI).

    Two siblings with delayed separation of the umbilical cord, recurrent skin ulceration and dental sepsis were shown to have defective neutrophil phagocytosis of opsonized yeast (S. cerevisiae) and respiratory burst to opsonized and unopsonized zymosan. Increased activity in the NBT reduction test, normal ingestion and killing of S. aureus, and normal spontaneous and directional motility were also demonstrated. These abnormalities of neutrophil phagocytosis were confined to the affected siblings; their healthy parents and brother showed normal neutrophil function. Both children had a polymorph neutrophil leucocytosis, and had normal humoral and cell-mediated immunity. SDS electrophoresis of neutrophil cell membrane preparations showed absence of a glycoprotein band of 175,000 daltons, which was present in the parents' neutrophils in reduced amounts. OKMI monoclonal antibody, which recognized the C3bi receptor (CR3) failed to bind to the affected siblings neutrophils. The findings in these children emphasize the importance of this receptor in phagocytosis, and possibly other neutrophil functions.
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9/14. pyoderma gangrenosum: clinical and laboratory findings in 15 patients with special reference to polyarthritis.

    Fifteen consecutive patients with PG have been studied during the period 1971-78. Systemic disease was found in 13 of the patients and preceded the skin disease in 10 patients by 1-25 years. Only two patients had ulcerative colitis. One patient had paroxysmal nocturnal hemoglobinuria and three patients had an IgA myeloma. Eight patients had polyarthritis; this was classical seropositive rheumatoid arthritis in two patients, and a seronegative inflammatory polyarthritis in six patients. Four patients had an unusual progressive erosive seronegative polyarthritis without evidence of granulomatous bowel disease, psoriasis, genital, urinary tract or eye disease. In three of these four patients the arthritis preceded the PG. synovial fluid analysis showed depressed complement levels and in one patient deposits of immunoglobulins and complement were demonstrated in the synovial membrane. The course of the arthritis was progressive with development of disabling joint deformities and erosive destruction of joints, despite treatment with penicillamine, corticosteroids and nonsteroidal anti-inflammatory drugs. One other patient had severe degenerative joint disease and chondrocalcinosis in association with a seronegative inflammatory polyarthritis, and another patient had ulcerative proctitis and severe degenerative joint disease secondary to chronic seronegative inflammatory polyarthritis. None of the patients had colitic arthritis, but in view of the association between PG and ulcerative colitis, some patients previously reported with PG and joint disease may have been suffering from the arthritis of ulcerative colitis. PG developed at the site of skin trauma in six patients. The natural history of the skin disease ran one of two courses: an acute, progressive course in which the ulcers rapidly enlarged until arrested by treatment; and a chronic course in which the lesions extended slowly and which after a period of weeks began to show signs of spontaneous healing. In only the patients with ulcerative colitis was there any correlation between the activity of the associated disease and the onset and progression of the skin disease. serum complement levels were normal and no circulating cryoprecipitable immune complexes were found. skin histology showed no evidence of vasculitis and direct immunofluorescence examination of involved skin was negative for IgG, IgM, IgA and C3. No consistent abnormality of cell-mediated immunity or neutrophil function was found and no significantly increased prevalence of any HLA antigen type was noted. Twelve patients have been treated with systemic corticosteroids. Six of these patients developed serious steroid complications and four patients have died, all from complications of steroid therapy.
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10/14. Cutaneous vasculitis complicating coeliac disease.

    A 38 year old female, with chronic uncontrolled coeliac disease, presented with the rare complication of cutaneous leucocytoclastic vasculitis. Detailed study failed to identify any cause for the vasculitis, other than the underlying coeliac disease. Haematuria and proteinuria with mesangial nephritis were also demonstrated on renal biopsy with electron microscopic study. It is speculated that exogenous or endogenous antigens permeated the abnormal small bowel mucosa leading to formation of circulating immune complexes. Subsequent tissue deposition of these complexes then resulted in vasculitis and nephritis. The skin lesions cleared completely after treatment with a strict gluten free diet.
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