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[Pyoderma gangrenosum and orbital pseudotumor].

Pyoderma gangrenosum belongs to the chronic, neutrophilic and necrotic dermatoses. These very painful, inflammable and purulent skin-ulcers often arise after injury or surgical intervention. About half of the patients suffer from systemic disease, for example hematological, gastrointestinal or rheumatic diseases. Our report includes a 77-year-old female with a multilocular emergence of pyoderma gangrenosum following surgery for an orbital pseudotumor. An additional lesion was detected on her anterior neck. Because the possibility of wound-infection was suspected, the lesion was treated locally and with a systemic antibiotic therapy. After this treatment the condition of the wound deteriorated. Upon dermatological examination, pyoderma gangrenosum was identified and a immunosuppressive therapy with corticosteroids and cyclosporin was initiated. As a result of this treatment the lesion healed slowly--with the formation of typical, atrophic and reticular scars. A connection between the pyoderma gangrenosum and the orbital pseudotumor can, on the basis of similar clinical and structural characteristics, not be excluded. A related disease was not diagnosed in this patient. Recurrence can be predicted in patients who have had skin injuries, therefore strict observation is required.

Aged↗

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↗

Multiple aseptic pulmonary nodules with central necrosis in association with pyoderma gangrenosum.

Pulmonary manifestations of pyoderma gangrenosum are relatively rare. We report the case of a 45-year-old patient with multiple pulmonary nodules with central necrosis as assessed by CT scan. The patient had a 4-year history of pyoderma gangrenosum with only minor skin manifestations. A CT-guided, fine-needle biopsy of the lung revealed a nonspecific, inflammatory, aseptic necrotic process, which was comparable to the skin biopsy of one pyoderma lesion. Following the initiation of oral prednisolone therapy, a rapid resolution of the pulmonary nodules occurred. We conclude that pulmonary nodules represent a rare pulmonary manifestation of pyoderma gangrenosum.

Female↗

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↗

Infliximab for treatment of pyoderma gangrenosum associated with inflammatory bowel disease.

OBJECTIVES: Pyoderma gangrenosum is an immune-mediated inflammatory condition characterized by ulcerative skin lesions affecting 1-2% of patients with inflammatory bowel disease (IBD). Treatment includes wound care, antibiotics, corticosteroids, and immunomodulators. However, response to therapy varies, and many patients with pyoderma gangrenosum have disease that is refractory to these agents. The aim of this study was to assess the response of medically refractory pyoderma gangrenosum to infliximab. METHODS: This was a multicenter retrospective study of patients with IBD and medically refractory pyoderma gangrenosum treated with infliximab. Data collected included the following: baseline demographics; duration of IBD; history of bowel resection; duration of skin lesions; number, size, and location of pyoderma gangrenosum lesions; prior medications; dose and number of infliximab infusions; bowel activity before and after infliximab; pyoderma gangrenosum activity before and after infliximab therapy; time to response and time to healing of pyoderma gangrenosum lesions; recurrence of pyoderma gangrenosum after infliximab; corticosteroid taper; and adverse reactions to infliximab. RESULTS: There were 13 patients with moderate to severe pyoderma gangrenosum and IBD treated with infliximab. All patients demonstrated complete healing of the skin lesions. Three patients had a complete response to induction infliximab therapy and did not require additional treatment. Ten patients responded to induction infliximab and have maintained pyoderma gangrenosum healing with infusions every 4-12 wk. All patients receiving corticosteroids were able to discontinue them completely after institution of infliximab treatment. Infliximab was well tolerated; the only treatment-related adverse events were sunburn in one patient and an infusion reaction in another. CONCLUSIONS: Infliximab is a safe and effective treatment for IBD-associated pyoderma gangrenosum.

Adult↗

Management of pyoderma gangrenosum.

Management of pyoderma gangrenosum continues to be a therapeutic challenge, both because the low incidence of the disease makes it difficult to conduct large-scale randomized studies and because pathogenic mechanisms are not yet well defined. The selection of drugs and dosing schedules is therefore mainly guided by clinical experience.

Cromolyn Sodium↗

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↗

Pyoderma gangrenosum: a great marauder.

Pyoderma gangrenosum is a progressively necrotizing and ulcerative skin disease that mimics a severe bacterial infection. However, the cause is not infectious in nature and the lesions are refractory to local wound care and antibiotic therapy. The etiology of pyoderma remains unknown, although pathogenic mechanisms may involve immunologically mediated cutaneous damage. The authors report a 67-year-old woman in whom a necrotic ulcer developed at a chest tube site. Treated with local wound care and antibiotics, this lesion spread progressively to involve 15% of her body surface area. A septic clinical picture developed despite sterile cultures, and she required several operative debridements. Her disease continued to spread and finally a diagnosis of pyoderma gangrenosum was considered. Treated with systemic steroids, hyperbaric oxygen (HBO), and local wound care, she eventually underwent skin grafts.

Aged↗

Pyoderma gangrenosum following breast reconstruction.

Pyoderma gangrenosum is an unusual cause of skin necrosis following surgery, particularly in those without an associated systemic condition. There have been reports of the condition in this context but not in relation to breast reconstruction. We present a case of pyoderma gangrenosum following latissimus dorsi flap reconstruction of a breast.

Breast Neoplasms↗