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Treatment of pyoderma gangrenosum with methotrexate.

Pyoderma gangrenosum is an ulcerative skin condition of unknown cause. It is often refractory to treatment, requiring high dosages of immunosuppressive medications. A patient with idiopathic pyoderma gangrenosum was successfully treated with methotrexate and weaned off steroids for the first time in four years. The patient had received multiple skin grafts and failed topical treatment, rifampin, dapsone, azathioprine, and intralesional steroids. She required prednisone 60 mg/day for lesion healing. Methotrexate was given orally, with rapid response, allowing discontinuation of prednisone seven months after beginning therapy. There is evidence of neutrophil dysregulation in pyoderma gangrenosum. Methotrexate has been shown to decrease neutrophil migration and chemotaxis, suggesting a role for its use in pyoderma gangrenosum.

Adult

Pyoderma gangrenosum: a review.

Pyoderma gangrenosum is a poorly understood disease characterized by exacerbations and remissions of morphologically unique skin ulcers. It frequently is thought to be the cutaneous manifestation of an underlying systemic disease. In this review of pyoderma gangrenosum, the characteristics of the disease are described and a differential diagnosis is formulated. Associations with systemic diseases are made through a review of the literature. The pathophysiology of pyoderma gangrenosum and probable causes are considered, and special consideration is given to the immunologic mechanisms that may be operative in the disease. Finally the currently available therapeutic alternatives are reviewed.

Colitis, Ulcerative

Pyoderma gangrenosum with myelofibrosis.

Pyoderma gangrenosum is a papulovesicular skin disorder commonly associated with underlying systemic disease, but rarely with the myeloproliferative syndromes. A case of rapidly progressive pyoderma is cited in a 77-year-old white man who had no other evidence of disease aside from macrocytic anemia. Bone marrow biopsy revealed proliferation of fibroblasts and a dense reticulin network consistent with myelofibrosis. Response of the pyoderma to steroid therapy was dramatic. Although pyoderma gangrenosum is more commonly associated with inflammatory bowel disease and rheumatoid arthritis, this is the fifth reported case of its coexistence with idiopathic myelofibrosis.

Aged

Pyoderma gangrenosum and myelodysplasia.

Pyoderma gangrenosum has been recognized occurring in association with acute and chronic leukaemia. More recently it has been described in patients with myelodysplasia (Jacobs, Palmer & Gordon-Smith 1985). We report a case of pyoderma gangrenosum in a patient with a preceding history of myelodysplasia, illustrating the problem of controlling the skin disease in this condition and the eventual transformation of the myelodysplasia into acute leukaemia. Treatment of the leukaemia resulted in improvement of the skin lesions.

Humans

Intralesional steroid therapy of pyoderma gangrenosum.

Treatment of pyoderma gangrenosum complicating ulcerative colitis has in the past been frustrating and usually unsuccessful, frequently resulting in colectomy. Intralesional steroid therapy has been used off and on in a few patients, but the treatment is not widely known to gastroenterologists. We report two patients successfully treated for early pyoderma gangrenosum by intralesional injection of triamcinolone acetonide (Kenalog, 40 mg injection) together with conventional doses of systemic steroids. A single series of injections was sufficient to bring about healing of the lesions in both patients. The treatment permitted early discharge of patients from the hospital, and the skin lesions were completely healed within 2 months. The lesions have not recurred in follow-up examinations of 11/2 and 21/2 years, respectively. We hope that other physicians will find this approach equally effective.

Adolescent

The evolution of pyoderma gangrenosum. A clinicopathologic correlation.

Pyoderma gangrenosum is a well-known clinical cutaneous condition, that has had a variety of conflicting microscopic descriptions. In an attempt to further our understanding of this condition, we obtained nine skin biopsy specimens of evolving, fully developed, regressing, and resolved lesions from six patients with pyoderma gangrenosum. We found that histopathologically, pyoderma gangrenosum evolves from folliculitis and abscess formation; it may also show leukocytoclastic vasculitis. The lesions then evolve to suppurative granulomatous dermatitis and finally regress with prominent fibroplasia. We illustrate the clinical and microscopic features herein and compare them to previous descriptions.

Adult

'Sticky' neutrophils, pathergic arthritis, and response to heparin in pyoderma gangrenosum complicating ulcerative colitis.

Pyoderma gangrenosum is strongly associated with inflammatory bowel disease and exhibits pathergy, occurring at sites of previous minor trauma. A patient is presented with a 21 year history of extensive ulcerative colitis, who developed pyoderma gangrenosum and arthralgia while receiving high dose corticosteroids for active ulcerative colitis. The arthralgia exhibited pathergy affecting particularly the left temporomandibular joint, which was stressed by an asymmetric bite, and the left elbow, which had been fractured many years previously. This prompted the hypothesis that neutrophils in this condition may be marginated, as a result of increased stickiness of either the neutrophil or the vascular endothelium. The introduction of heparin therapy was associated with rapid resolution of the arthralgia, pyoderma gangrenosum, and ulcerative colitis.

Adult

Pyoderma gangrenosum--a literature review.

Pyoderma gangrenosum is an ulcerative skin disorder with typical clinical characteristics. Histologic and laboratory findings are nonspecific. Pyoderma gangrenosum is associated with internal disorders including inflammatory bowel disease, paraproteinemias, leukemias, and arthritis. The pathogenesis of pyoderma gangrenosum is unknown, although a partial defect of cell-mediated immunity may exist. Treatment includes bedrest, local care, sulfonamides, sulfones, and corticosteroids.

Adolescent

Pyoderma gangrenosum at surgery sites.

Pyoderma gangrenosum is a necrotizing and ulcerative skin disorder often associated with underlying systemic diseases. The etiology remains obscure, with recent investigations emphasizing an altered immune system. A case report is presented of pyoderma gangrenosum occurring at surgical sites in the absence of predisposing factors. Pyoderma gangrenosum in this setting can mimic infectious causes of wound necrosis. Early recognition of the characteristic lesion morphology may prevent unnecessary treatment directed toward infectious agents and facilitate effective control with systemic corticosteroid therapy.

Coronary Artery Bypass

Pyoderma gangrenosum in childhood.

Pyoderma gangrenosum is an uncommon cutaneous disorder that often begins as a small pustule, but results in localized skin destruction. The latter is characterized by an expanding ulceration with undermined violaceous borders. It may be due to an altered immune response. It often, but not invariably, reflects underlying systemic disease.

Child

Pyoderma gangrenosum and chronic persistent hepatitis.

Pyoderma gangrenosum is a diagnosis of exclusion. A 36-year-old woman was clinically diagnosed as having pyoderma gangrenosum and appropriate exclusion tests were performed. Pyoderma gangrenosum may occur in association with underlying diseases such as leukemia, monoclonal gammopathy, inflammatory bowel disease, arthritis, and chronic active hepatitis. However, these diseases were excluded in this patient who did have chronic persistent hepatitis. To our knowledge, this is the first reported case of chronic persistent hepatitis and pyoderma gangrenosum.

Adult

Pyoderma gangrenosum in infants and children.

Pyoderma gangrenosum is an uncommon ulcerative skin disorder that occurs in all age groups. Approximately 4% of patients are infants and children. There are several notable differences between the childhood and adult manifestations of the disease, including the distribution of lesions and associated disorders. We reviewed the childhood cases (< or = 18 yrs of age) of unequivocal pyoderma gangrenosum in the English literature and tabulated the trends in clinical features, associated disorders, and therapy. We report our 3-week-old patient, the youngest documented case. Of the 46 patients, only 4 were less than 1 year of age. A systemic illness was present in 74% of the older children, most commonly, ulcerative colitis. Only one infant had an associated problem (HIV+) at the time of onset. Infants appear to have an unusual distribution of perianal and genital lesions not often described in other age groups. Our review suggests that pyoderma gangrenosum in children has a similar clinical appearance to that in adults. It is associated with some of the same underlying disorders, but with different frequencies. The distribution of lesions in children is similar, often involving the lower extremities, but pyoderma gangrenosum of the head and face appears to be more common in children. Infants may have ulcers in genital and perianal areas. The most frequently prescribed treatment for children is systemic corticosteroids, which generally are very effective.

Adolescent

SAPHO syndrome and pyoderma gangrenosum: is it fortuitous?

Pyoderma gangrenosum is well known as an associated feature of inflammatory bowel disease (IBD). Recently, higher than normal prevalence of IBD in patients with the SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome has been reported. However, the association of pyoderma gangrenosum with SAPHO syndrome without definitely excluded IBD has not been reported. We describe a case that suggests a possible connection between these 2 entities.

Acne Vulgaris

Cyclosporine for pyoderma gangrenosum.

A case of pyoderma gangrenosum that occurred in the absence of underlying illness and was initially misdiagnosed is described. Hemorrhagic pustules and ulcers appeared over vein-graft sites after open heart surgery and were treated with cyclosporine. The result was rapid and complete resolution. It is the purpose of this report to emphasize pyoderma gangrenosum as a cause of ulceration in healthy individuals and to highlight the addition of cyclosporine to the therapeutic armamentarium.

Cyclosporine

Pyoderma gangrenosum associated with primary thrombocythaemia.

Pyoderma gangrenosum is most commonly associated with inflammatory bowel disease and rheumatoid arthritis, but it has been associated with various haematological malignancies. A 54-year-old man with no history of bowel disease or arthritis presented with a leg ulcer, which healed after treatment. Results of bone marrow aspiration were compatible with primary thrombocythaemia. Seven weeks later there were pronounced recurrence of the ulceration and pyoderma gangrenosum was diagnosed. The appearance of pyoderma gangrenosum associated with blood disorders may differ from that associated with bowel and joint disease.

Humans

Recurrent pyoderma gangrenosum and agnogenic myeloid metaplasia.

Pyoderma gangrenosum has been associated with myelogenous leukemia and plasma cell dyscrasia. When associated with leukemia, pyoderma gangrenosum often has a distinctive clinical presentation with an advancing bullous margin. The pathogenesis of this disorder is unknown, although defective immune mechanisms may be operative. The occurrence of pyoderma gangrenosum and agnogenic myeloid metaplasia in the same patient has now been reported sufficiently to make it a recognized association.

Female

Atypical pyoderma gangrenosum with leukemia.

Pyoderma gangrenosum (PG) has been increasingly reported in association with myeloproliferative disorders. Monoclonal gammaopathy, myeloma, myeloid metaplasia, and polycythemia have all been found in association with PG. Recently, seven cases of PG in association with leukemia have been described: three cases with acute myeloblastic leukemia, two cases with chronic myelogenous leukemia, one case with acute lymphoblastic leukemia, and one case with acute leukemia of either plasma cell or myeloblast origin. To these we add two cases of PG with acute myeloblastic leukemia. These patients often have an atypical clinical presentation for PG, with bullae and relatively superficial involvement obscuring the correct diagnosis.

Acute Disease