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Topical treatment with 1% sodium cromoglycate in pyoderma gangrenosum.

BACKGROUND: Pyoderma gangrenosum is an uncommon skin disease usually treated with systemic drugs, most frequently corticosteroids. Isolated topical treatment is usually considered unsatisfactory. OBJECTIVE: To evaluate the effect of topical 1% sodium cromoglycate solution on pyoderma gangrenosum in 5 patients 25-30 years of age. METHODS: 5 patients, including 2 under systemic steroid treatment, hospitalized in our department from 1992 to 1993 because of pyoderma gangrenosum, were treated with topical 1% sodium cromoglycate solution. Systemic corticosteroids were further added in 2 patients whose initial improvement was inadequate. RESULTS: Initial improvement was noted in all 5 patients after 3-7 days of sodium cromoglycate treatment. Complete healing of the ulcers occurred within 5-8 weeks. CONCLUSIONS: Topical treatment with sodium cromoglycate can be effective as adjunctive or sole treatment in pyoderma gangrenosum.

Administration, Topical↗

Pyoderma gangrenosum and malignant pyoderma in Nigeria.

Five patients (four black Nigerian males and one Polynesian) with pyoderma gangrenosum (PG) were seen between May 1974 and March 1984, at the Ahmadu Bello University Teaching Hospital, Kaduna, Nigeria. Their age range was 12-42 years (mean 25.6 years). The female expatriate Polynesian patient had PG localized to the upper back while the other four patients had severe and extensive PG lesions. Local cleansing and dressing of the ulcers combined with appropriate systemic antibiotics produced healing in two of these patients. The empirical addition of dapsone and rifampicin led to complete healing in two others, but only transient remission in one patient who, after 22 years of disease activity, died at home from an aggressive and accelerated form of the disease best described as 'malignant pyoderma' gangrenosum.

Adult↗

Chlorambucil is an effective corticosteroid-sparing agent for recalcitrant pyoderma gangrenosum.

BACKGROUND: Pyoderma gangrenosum (PG) may fail to respond to corticosteroids. Immunosuppressive and cytotoxic agents are useful in patients with recalcitrant disease. We describe our experiences with chlorambucil for PG. OBJECTIVE: Our purpose was to evaluate the effectiveness of oral chlorambucil in patients with PG recalcitrant to treatment with prednisone, immunosuppressive therapy, or both. METHODS: Six patients with recalcitrant PG were given oral chlorambucil 2 to 4 mg/day. Four patients were treated with a combination of prednisone and chlorambucil, and two received chlorambucil alone. Response was based on (1) a diminution in the size of the ulcers, or their complete healing, or (2) a decrease in the dose of corticosteroid therapy. RESULTS: Beneficial effects were noted within 6 to 8 weeks in all six patients, and corticosteroids were eventually discontinued in all patients. Currently only two patients are still receiving chlorambucil; the other four stopped chlorambucil after 6 to 24 months of treatment. Their disease has remained in remission for 4 to 9 years. Relapse of disease occurred within 1 to 4 months after stopping therapy in one of the two remaining patients or reducing the dose in the other. In both patients, the disease is again responding to treatment. Minimal chlorambucil toxicity has been noted, consisting of leukopenia in one patient. CONCLUSION: Our findings suggest that chlorambucil is an effective corticosteroid-sparing agent for the control of PG.

Adult↗

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↗

Clinical features and treatment of peristomal pyoderma gangrenosum.

CONTEXT: Peristomal pyoderma gangrenosum (PPG), an unusual variant of pyoderma gangrenosum, has been reported almost exclusively in patients with inflammatory bowel disease (IBD) and is frequently misdiagnosed. OBJECTIVE: To better characterize the clinical manifestations, diagnosis, and management of PPG. DESIGN, SETTING, AND PATIENTS: Retrospective analysis of 7 patients with PPG observed in a university-affiliated community setting between 1988 and December 1999. MAIN OUTCOME MEASURES: Clinical and histopathologic features, associated disorders, and microbiologic findings. RESULTS: Two patients had Crohn disease, 2 had ulcerative colitis, and 3 had abdominal cancer. Five patients had at least 1 relapse of PPG after initial healing. Although 3 of 4 patients with IBD had active bowel disease, a parallel course with PPG occurred in only 1 patient. Both patients whose stoma was relocated developed an ulcer at the new site. Effective therapies included topical superpotent corticosteroids; intralesional injection of triamcinolone acetonide at the ulcer margin; topical cromolyn sodium; oral dapsone, prednisone, cyclosporine, mycophenolate mofetil; and intravenous infliximab. CONCLUSION: Our experiences demonstrate that although PPG has been most often reported in patients with IBD, it may occur in the absence of IBD. Biopsy of the skin lesion is not diagnostic but excludes other causes. Relocation of the stoma may be associated with a new ulceration and should be avoided. Trauma to the skin of a predisposed patient may elicit the pustules or ulcerations associated with pathergy. JAMA. 2000;284:1546-1548.

Adult↗

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↗

Recalcitrant pyoderma gangrenosum treated with thalidomide.

Pyoderma gangrenosum is a painful, noninfectious, ulcerating skin disorder often associated with systemic disease. Thalidomide has been used to treat many inflammatory dermatologic conditions and has been reintroduced in the United States to treat immune-modulated diseases such as pyoderma gangrenosum. The patient described, a 47-year-old man, had histologically confirmed pyoderma gangrenosum that did not respond to treatment with several courses of methylprednisolone. The ulcer healed with 10 weeks of oral thalidomide administration.

Anti-Inflammatory Agents↗

Severe peristomal pyoderma gangrenosum: a case study.

Pyoderma gangrenosum is an autoimmune disease that often manifests itself as painful ulcers. When these ulcers occur in the peristomal area, symptom management and wound care must be balanced against the need to pouch the stoma. Although the treatment of pyoderma gangrenosum is not standardized, systemic steroid therapy is frequently used as first-line therapy. The WOC nurse is often asked to manage both the pouching needs and topical therapy of the patient with peristomal pyoderma ulcerations. This article describes the management of severe pyoderma gangrenosum ulcerations in a 59-year-old woman with a long-standing ileostomy.

Bandages↗

'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↗

Koebner phenomenon in an ANCA-positive patient with pyoderma gangrenosum.

A male with pyoderma gangrenosum is reported. The clinical and histological features were typical. The initial lesions resolved with characteristic cribriform scars. A few days after the complete recovery, he developed several necrotizing focal lesions localized to the scarred areas. A further histological examination revealed a granulation tissue rich in neutrophils and signs of necrotizing vasculitis. We found a high titer of circulating perinuclear antineutrophil antibodies (p-ANCA), which are a serological marker for various systemic diseases. An immunological circulating factor has been repeatedly suggested to be the "primum movens" of pyoderma gangrenosum. We discuss the unusual clinical presentation interpreted as a Koebner phenomenon and the possible role of immune factors in enhancing circulating-endothelial cell interactions in relation to the pathogenesis of pyoderma gangrenosum.

Antibodies, Antineutrophil Cytoplasmic↗

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↗