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Dramatic improvement of pyoderma gangrenosum with infliximab in a patient with PAPA syndrome.

Infliximab, a chimeric antitumor necrosis factor alpha monoclonal antibody (anti-TNF alpha), has been recently shown to have a beneficial effect on pyoderma gangrenosum associated with inflammatory bowel disease. Patients with the syndromic triad of pyogenic sterile arthritis, pyoderma gangrenosum, and acne, an autoinflammatory process caused by mutations in the CD2 binding protein-1 (CD2BP1) gene, can have severe pyoderma gangrenosum. We describe a 14-year-old patient with this syndrome who was unresponsive to multiple therapies. A dramatic improvement in his pyoderma gangrenosum was observed after one infusion of infliximab, and a second infusion led to its resolution. Our observation extends the therapeutic use of infliximab to this component of PAPA syndrome.

Acne Vulgaris↗

Wall-eyed bilateral internuclear ophthalmoplegia (Webino syndrome) and myelopathy in pyoderma gangrenosum.

A 35-year-old female with pyoderma gangrenosum developed paraparesis with a sensory level at L1. Three months later she complained of diplopia and was found to have bilateral internuclear ophthalmoplegia with exotropia and no ocular convergence. The term Webino syndrome has been coined to design this set of neuro-ophthalmologic findings. Although it was initially attributed to lesions affecting the medial longitudinal fasciculus and the medial rectus subnuclei of the oculomotor complex in the midbrain the exact location of the lesion is still disputed. In the present case both myelopathy and Webino syndrome were probably due to vascular occlusive disease resulting from central nervous system vasculitis occurring in concomitance to pyoderma gangrenosum.

Adult↗

Pyoderma gangrenosum after coronary artery bypass grafting.

Pyoderma gangrenosum is a rare cutaneous disorder that may complicate recovery after a cardiac operation. The lesions appear to represent a necrotizing infection; however, they do not respond to surgical debridement and antibiotic therapy. The treatment is based on high-dose corticosteroids and concomitant treatment of any underlying systemic disease.

Aged↗

Successful treatment of pyoderma gangrenosum with topical 5-aminosalicylic acid.

Pyoderma gangrenosum in a twenty-nine-year-old woman with associated long-standing Crohn's disease was treated with topical 5-aminosalicylic acid cream. Significant improvement in the ulceration occurred despite progressive deterioration of the inflammatory bowel disease. The possible mode of action is reviewed. The efficacy, together with the lack of side effects, warrants further study into the therapeutic role of topical 5-aminosalicylic acid.

Administration, Cutaneous↗

Systemic pyoderma gangrenosum responding to infliximab and adalimumab.

Pyoderma gangrenosum (PG) is frequently associated with constitutional symptoms as part of a nonspecific inflammatory response. However, extracutaneous discrete aseptic neutrophilic infiltrates are extremely rare. We report a patient with idiopathic PG with splenic and psoas muscle involvement. His disease was extremely aggressive and was unresponsive to conventional immunosuppressive therapy. His cutaneous and extracutaneous PG cleared with infliximab and adalimumab.

Adalimumab↗

Pyoderma gangrenosum: an unusual cause of periaural ulceration.

A case of pyoderma gangrenosum affecting the pinna and neck of a diabetic patient is reported. Appearances were suggestive of malignant otitis externa. A small biopsy resulted in rapid and aggressive exacerbation of the lesion. Pyoderma gangrenosum, although uncommon, should be considered as a cause of ulceration, particularly when the ulcer yields no growth on culture in a non-dependent area.

Diabetes Complications↗

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.

Aged↗

Pyoderma gangrenosum associated with Takayasu's arteritis.

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis characterized by destructive, necrotizing and noninfective ulceration of the skin mostly on lower extremities. PG is well known as a complication of Takayasu's arteritis in Japan. However, this association is not commonly observed in North American and European patients. We describe a case of PG that was associated with Takayasu's arteritis who was successfully treated with systemic cyclosporin. We have reviewed 35 well-documented PG cases with Takayasu's arteritis in comparison to 106 PG cases without Takayasu's arteritis. The results demonstrate that this association occurs predominantly in young females and that these cases exhibit more widespread PG lesions.

Adult↗

Treatment of Pyoderma gangrenosum with low-dose colchicine.

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis of unknown origin. Systemic agents occasionally administered provide either incomplete long-term control of the disease or have been associated with serious adverse side effects after chronic administration. We present two patients with PG successfully treated with low-dose colchicine. Antimitotic, anti-inflammatory and immunomodulating properties of colchicine might account for its beneficial effects in PG patients. Colchicine is effective and well tolerated in low doses by most patients. In addition, it is inexpensive and safer for long-term treatment than corticosteroids and other immunosuppressive agents. Colchicine may be proposed either as a single agent or as a corticosteroid-sparing agent for early treatment of PG.

Adult↗

Pyoderma gangrenosum: associations revisited.

Fourteen cases of pyoderma gangrenosum were seen over a period of 24 years at the Hull Royal Infirmary Dermatology Department. Several associated conditions were found. Seven cases were associated with rheumatoid arthritis of which five were sero-positive, including one with Felty's syndrome. One case was associated with both ulcerative colitis and psoriasis; one with polycythemia rubra vera; two patients had diverticular disease including one who also had rheumatoid arthritis; one had positive syphilis serology. In three cases there was no significant associated disease identified. Ten out of the fourteen cases were women, indicating a female preponderance by a ratio of about 2F:1M; a figure similar to that stated by Seitzinger. The age of presentation ranged from 30 to 80 years.

Adult↗

Giant cells in pyoderma gangrenosum.

It has been claimed that pyoderma gangrenosum (PG) lesions may contain granulomatous foci when associated with Crohn's disease. To test this assertion, we obtained clinical histories and archived cutaneous biopsies from 34 PG patients. Thirteen of these patients had inflammatory bowel disease (IBD). Immunostaining with PGM1, a macrophage marker, revealed well-formed giant cells with three or more nuclei in biopsies from 6 of 13 patients with IBD. Five of the 6 biopsies came from patients with Crohn's disease and one from a patient with ulcerative colitis. Two were peristomal. In the 21 patients who had PG without IBD, no giant cells were seen. Thus, PGM1+ histiocytic giant cells within a PG lesion may be indicative of associated IBD (p = 0.006), particularly Crohn's disease.

Crohn Disease↗

Atypical pyoderma gangrenosum after breast reduction.

Although rare, pyoderma gangrenosum (PG) occurs as one of the worst local complications following plastic surgery to the breast. The early manifestations are similar to a necrotizing wound infection, so diagnosis and correct management are often delayed. Failure of response to the aggressive treatment strategies needed for necrotizing wound infections and the phenomenon of pathergy will often raise the clinical suspicion of PG. The main steps of therapy consist of minimal wound debridement and initiation of corticosteroids and/or immune modulation. Repair of skin defects requires care and attention. Smaller defects are best left to heal by secondary intention, as pathergy can reactivate the syndrome and cause an even larger skin wound. Larger defects are repaired with skin grafts or flaps. We report a case of atypical (bullous) PG in a healthy 57 year-old white woman following reduction mammaplasty.

Breast Diseases↗

Pyoderma gangrenosum affecting the foot. A case report.

The diagnosis of pyoderma gangrenosum is a difficult one to make because of the condition's ability to mimic other ulcerative lesions of the foot and its lack of specific laboratory and pathologic findings. A high index of clinical suspicion can lead to a definitive diagnosis. This article presents a case report and a discussion of the evaluation and management of pyoderma gangrenosum.

Adrenal Cortex Hormones↗

Pyoderma gangrenosum and progressive cutaneous ulceration.

Pyoderma gangrenosum is a cutaneous disorder characterized by slowly progressive ulceration which is refractory to local wound care and antibiotic therapy. The diagnosis is made after the exclusion of other causes of cutaneous ulceration. The cause of pyoderma is thought to be a deficiency in host immune reactivity, and a systemic illness is present in 80% of patients. High-dose corticosteroids and appropriate treatment of the underlying disease represent the mainstays of therapy, although hyperbaric oxygen has recently shown promise in the healing of skin grafts over these lesions.

Adult↗

Pyoderma gangrenosum: a challenging complication of bilateral mastopexy.

A case of pyoderma gangrenosum progressively developing after bilateral mastopexy at the surgical site is described. The described case was successfully treated with corticosteroids, the application of the dermal regeneration template Integra and autologous skin grafts. This approach was able to save the patient's life and to generate a high-quality aesthetical outcome. The article reported the case, reviewed the literature of pyoderma gangrenosum related to mastopexy or augmentation mammoplasty and discussed the use of a dermal regeneration template to optimise aesthetical results after reconstructive surgery.

Adult↗

Pyoderma gangrenosum responsive to minocycline hydrochloride.

Pyoderma gangrenosum (PG) is a characteristic ulcerative condition of unknown etiology. Four cases are reported of patients with PG who responded to minocycline hydrochloride therapy. Possible mechanisms for this apparently efficacious modality are discussed.

Adult↗

Erythema nodosum progressing to pyoderma gangrenosum as a complication of Crohn's disease.

Erythema nodosum occurs more frequently than pyoderma gangrenosum in Crohn's disease. We report the occurrence of both lesions in a woman with Crohn's disease and the transformation of one to the other. Pyoderma gangrenosum has been described at the site of previous trauma in Crohn's disease and at the site of other skin conditions in other circumstances, but, to the best of our knowledge, actual progression from erythema nodosum to pyoderma gangrenosum has been reported on only one previous occasion in Crohn's disease.

Adult↗