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PubMed1995

'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↗

PubMed1997

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↗

PubMed1978

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↗

PubMed1985

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↗

PubMed2003

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↗

PubMed2001

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↗

PubMedDate not supplied

Pyoderma gangrenosum: uncommon presentations.

Pyoderma gangrenosum (PG) is a rare, inflammatory, noninfective, nonneoplastic skin disorder, which is often associated with systemic diseases. These include inflammatory bowel disease, rheumatoid arthritis, paraproteinaemia, or hematologic malignancy, which can be found in up to 50% of patients with some variants of PG. Brunsting et al (Arch Dermatol 1930;22:655-80) first described PG as a disease entity in 5 patients who had painful, enlarging necrotic ulcers with bluish undermined borders surrounded by advancing zones of erythema. Four of these patients had chronic ulcerative colitis. They felt that the condition might be associated with bacterial infection (pyoderma) and considered it as linked to the underlying bowel disease. Although the cause of PG remains obscure, bacterial infection seems to be unrelated to its causation, rendering the term pyoderma redundant. In addition, the number of conditions reported in association with PG has markedly expanded in recent years, showing clearly that this is not solely a cutaneous manifestation of inflammatory bowel disease. The clinical concept of PG has also been broadened, and certain clinical variants of PG have been linked with different types of associated disease seen in these patients.

Adult↗

PubMed1996

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↗

PubMed1985

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↗

PubMed2004

Successful treatment of pyoderma gangrenosum with topical 0.5% nicotine cream.

Pyoderma gangrenosum is potentially a devastating and destructive disorder. There is no uniformly effective or specific therapy for pyoderma gangrenosum. Previous reports of nicotine therapy for pyoderma gangrenosum have suggested it to be efficacious. Unfortunately, previous reports were restricted by the use of commercially available preparations of nicotine, either as a gum or patch formulation. We have used topical nicotine 0.5% w/w cetamacrogol formula A cream that enables direct application onto the lesion, as well as dose and concentration variation. Two patients with pyoderma gangrenosum treated with topical nicotine 0.5% w/w cetamacrogol formula A cream are described here, both of whom had dramatic clinical resolution of their pyoderma gangrenosum.

Administration, Cutaneous↗

PubMed1994

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↗

PubMed2002

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↗

PubMed2000

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↗

PubMed1996

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↗

PubMed1993

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↗

PubMed2002

Successful treatment of postoperative pyoderma gangrenosum with cyclosporin.

Postoperative pyoderma gangrenosum (PPG), also known as postoperative progressive gangrene of Cullen, is a rare and rapidly evolving complication of surgical procedures. Since the first description by Cullen in 1924 (Surg Gynecol Obstet 1924; 38: 579-582) various case reports have been published. Even in typical cases PPG is often misdiagnosed and therefore wrongly treated; the unknown aetiology makes treatment difficult. The therapies used for pyoderma gangrenosum include systemic corticosteroids, azathioprine, dapsone, mercaptopurine, sulphasalazine, sulphapyridine, thalidomide, cyclophosphamide, clofazimine, isotretinoin, immunoglobulins and cyclosporin. We report on two patients with PPG following breast surgery who were successfully treated with low-dose cyclosporin (2.5-5 mg/kg per day).

Adult↗

PubMed2006

A case of vulvar pyoderma gangrenosum associated with collagenous colitis.

Pyoderma gangrenosum is a reactive inflammatory dermatosis which belongs to the spectrum of neutrophilic dermatoses. Due to a lack of diagnostic criteria, pyoderma gangrenosum is mainly a diagnosis of exclusion. It is rarely observed on the perineum, and vulvar involvement is even less frequent. Collagenous colitis is an idiopathic inflammatory colonic disease that is included in the microscopic colitides. The colonic mucosa and the crypt architecture are preserved but histologic alterations are found. We describe a case of collagenous colitis associated with vulvar pyoderma gangrenosum that improved spectacularly with cyclosporine 3 mg/kg/day and the twice-daily application of topical tacrolimus 0.1%.

Adult↗

PubMed1998

Pyoderma gangrenosum producing saddle nose deformity.

Pyoderma gangrenosum affecting the nose is rare and this may lead to diagnostic confusion because of the large differential diagnosis. As diagnosis is made, largely, on the basis of exclusion the treatment of pyoderma gangrenosum may be unduly delayed. The condition is often disfiguring, particularly following inappropriate surgical intervention, and early diagnosis is therefore important. We present a case of pyoderma gangrenosum managed initially in the community with minor surgery and resulting in the rare complication of saddle nose deformity.

Aged↗