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Pyoderma gangrenosum of the "sinus mammarum" in ulcerative colitis.

The first part of this article deals with the report of a patient suffering from pyoderma gangrenosum of the "sinus mammarum" associated with asymptomatic ulcerative colitis. This is followed by a revision of the present epidemiological, etiological, pathogenetic and clinical knowledges about this systemic manifestation of chronic phlogosis of the colon. The Authors have analysed the treatment for this condition and emphasized the resistance of the cutaneous ulcer encountered to conventional medical therapy of the underlying colonic disease which proved to be efficacious only on the latter; this led to integrate traditional treatment with the use of perilesional injections of small doses of calcic heparin as an alternative to immunosuppressive drugs or surgery. Topical antithrombotic treatment, which can be justified by the histological findings of phenomena of the vasculitis in the edge of pyoderma gangrenosum, demonstrated to be crucial and represents a peculiarity in the case here reported, which is unique in the literature as far as the Authors know, since it has not been experimented by anyone else.

Adult↗

Pyoderma gangrenosum in immunosuppressed patients.

Two cases with pyoderma gangrenosum are presented. The course, in both cases, suggested that immunosuppressive therapy may play an etiological role in the disease. The first was a kidney recipient receiving prednisone and azathioprine and the second, a patient with pemphigus vulgaris who was treated with prednisone and methotrexate.

Adult↗

Pyoderma Gangrenosum and rheumatoid arthritis.

Two patients had pyoderma gangrenosum and rheumatoid arthritis. Biopsy specimens of the cutaneous ulcers in the patients showed a necrotizing vasculitis. Complement (C3) and immunoglobulins were not detected in the skin lesions. There was no important impairment of cell-mediated immunity observed.

Aged↗

Leukocyte chemotaxis and pyoderma gangrenosum.

Leukocyte chemotaxis (in five patients with pyoderma gangrenosum) was studied using a modification of the Boyden chamber method. In all patients the chemotactic response was significantly lower than in the controls. This abnormal chemotaxis was a result of an intrinsic neutrophil dysfunction. No significant difference was detected between the chemotactic response of leukocytes from patients with minimal or no skin involvement and those from patients with extensive lesions.

Adult↗

Fatal pyoderma gangrenosum in association with C7 deficiency.

Although pyoderma gangrenosum (PG) is often associated with systemic diseases, it has not been reported in association with congenital complement deficiencies. We describe an aggressive and ultimately fatal case of PG in a patient with a congenital C7 deficiency. Deficiencies of C7 can be associated with decreased neutrophil chemotaxis, phagocytosis, and opsonization, similar to the immunologic abnormalities described in patients with PG. Our patient's decreased complement level, if not directly related to the development of PG, may have contributed to the aggressive nature of her disease.

Adult↗

[Pyoderma gangrenosum and hemopathies. Apropos of 2 cases].

Pyoderma gangrenosum (PG) is an uncommon ulcerative disease of the skin. The cause is unknown but the condition is often associated with other diseases such as rheumatoid arthritis, ulcerative colitis, Crohn's disease or monoclonal gammopathy. The association between PG and haematological malignancies (acute leukaemia, Myeloproliferative disorders) is infrequent. Two cases of PG associated with haemopathy are described; one had primary thrombocythaemia and the other, acute myeloblastic leukaemia following for myeloma. The significance of this association is discussed in the light of other observations previously reported in the literature.

Adrenal Cortex Hormones↗

Aberrant neutrophil trafficking and metabolic oscillations in severe pyoderma gangrenosum.

Having previously associated metabolic oscillations with cell locomotion, we hypothesized that patients with abnormalities in neutrophil trafficking may display aberrant intracellular oscillations. A pyoderma gangrenosum patient exhibiting aberrant leukocyte trafficking in vivo and skin ulceration without infection was identified. This patient's neutrophils constitutively overexpressed and clustered the leukocyte integrins CR3 and CR4 and failed to display appropriate integrin-to-GPI receptor interactions. Increased levels of tyrosine phosphorylation were observed. NAD(P)H oscillations, which are sinusoidal in normals, were chaotic with multiple frequency components in this patient's neutrophils. Normal cell shape and sinusoidal NAD(P)H oscillations were restored by providing a pulsed electric field to drive metabolic oscillations and by temperature reduction. N-acetyl-D-glucosamine disrupted CR3 clusters and sinusoidal NAD(P)H oscillations returned. Anecdotal reports suggest that local hypothermia is clinically useful for this patient. These data define the first metabolic oscillation-associated disease and suggest that pyoderma gangrenosum can be classified as a dynamical disease at the cellular level.

Acetylglucosamine↗

[Pyoderma gangrenosum and pelvispondylitis without patent intestinal disease. Apropos of a case].

The authors report a case of pyoderma gangrenosum associated with a positive B27 pelvispondylitis. The search for a hemorrhagic rectocolitis, possible link between the two syndromes, revealed to be negative. Pelvispondylitis is an unusual complication of pyoderma gangrenosum. Review of the literature shows only one case of sacroiliitis, beside seronegative polyarthritis unrelated to HLA B27.

Adult↗

Pyoderma gangrenosum: a Waikato experience.

Ten cases of pyoderma gangrenosum were seen over a period of nine years at the Waikato Hospital dermatology clinic. Several associated conditions were found. Four cases were associated with inflammatory bowel disease, three cases with arthritis, one case was found in association with myelofibrosis. Four patients were diabetic, an incidence higher than in previous studies. Most patients were treated with oral steroids but additional therapies were used to achieve disease control and for their steroid-sparing effect.

Adult↗

Pyoderma gangrenosum as a complication of coronary artery bypass grafting.

A 60-year-old male patient developed progressive wound ulcerations, simulating wound sepsis after coronary bypass operation. The condition did not respond to intensive antibiotic therapy. Based on clinical signs and biopsy, the diagnosis of pyoderma gangrenosum was made and successfully treated with cyclosporin A. Pyoderma gangrenosum, although extremely rare, must be considered as a possible diagnosis in wound complications unresponsive to traditional therapy.

Angina Pectoris↗

Cyclosporine, azathioprine and local therapy for pyoderma gangrenosum.

A markedly obese 54 year old woman with seropositive rheumatoid arthritis, anaemia, dyspepsia, controlled hypothyroidism and depression presented with a seven month history of large pyoderma gangrenosum ulcers on the shins. Routine dressings for the ulcers had been ineffective. Her arthritis was being treated with azathioprine and NSAID's. Initial treatment with clobestasol propionate and disodium cromoglycate under occlusion produced only partial healing. Introduction of Cyclosporin A and continuation of topical therapy, with the addition of triamcinolone acetonide injections, led to progressive healing which was complete after seven months. There has been no relapse to date. Cyclosporine can be combined with azathioprine and local therapy for successful treatment of pyoderma gangrenosum.

Administration, Topical↗

Pyoderma gangrenosum of the breast treated with low-dose cyclosporin A.

Pyoderma gangrenosum (PG) is a painful chronic ulcerative skin disorder often occurring in association with systemic disease. It typically affects the lower limbs, but may also involve other sites, or sometimes develop after trauma of surgical procedures. We report the case of a woman with rheumatoid arthritis who developed disfiguring and severe PG of the right breast, a rare site, following biopsy for a benign breast lesion, and who was subsequently successfully treated with low-dose cyclosporin A.

Antirheumatic Agents↗

Crohn's disease of the penis masquerading as pyoderma gangrenosum: a case report and review of the literature.

Both pyoderma gangrenosum (PG) and cutaneous (metastatic) Crohn's disease (CCD) may occur in the setting of inflammatory bowel disease (IBD). Clinical distinction between PG and CCD may be difficult because clinical and pathologic features often are similar. Although surgical debridement is therapeutic in CCD, it may lead to increased tissue loss and disease progression (pathergy) in PG. Thus, it is important to determine a definitive diagnosis before surgical debridement, especially in tissue-sensitive sites. We present a patient with chronic ulceration of the penis who ultimately was diagnosed with CCD following an initial misdiagnosis of PG.

Crohn Disease↗

Pyoderma gangrenosum with a contiguous erosion of the distal ulna.

BACKGROUND: Pyoderma gangrenosum (PG) has been described in association with sterile neutrophilic infiltration of several organ systems, including the skeleton. The most frequent cause of bony disease in PG has been chronic, recurrent, multifocal osteomyelitis, a sterile inflammatory disease of children and young adults mimicking infectious osteomyelitis. Bony erosions have been only rarely described in direct contiguity to a PG ulcer. OBJECTIVE: We report a 53-year-old woman with recurrent PG who developed an erosion of the distal ulna adjacent to a PG ulcer. The patient responded to high-dose prednisone, and a repeat radiograph of the wrist four months later was normal. CONCLUSION: This case demonstrates another example of cortical osteolysis directly adjacent to a PG ulcer in which the bony changes may be neutrophil-induced.

Bone Diseases↗

Treatment of pyoderma gangrenosum with oral Tripterygium wilfordii multiglycoside.

Two patients with refractory pyoderma gangrenosum (PG) were treated with oral Tripterygium wilfordii multiglycoside (TWG). TWG is a Chinese medicine extracted from a medicinal herb, Tripterygium wilfordii Hook F, and has potent anti-inflammatory and immunosuppressive effects. The effect of TWG on PG was demonstrated by clinical findings. Improvement of the lesions occurred within two weeks, and the ulcers healed about a month. Mild side effects such as gastrointestinal disturbances were observed in both patients. These side effects were patient-acceptable, and there was no need to stop the treatment. Transient elevation of serum ALT was observed in one patient; the serum ALT returned completely to normal after the discontinuation of TWG. These results suggest that TWG may be an effective alternative for refractory PG and that careful monitoring of liver function during TWG treatment is necessary.

Administration, Oral↗

Pyoderma gangrenosum. Response to cyclophosphamide therapy.

A 46-year-old woman had a 17-year history of intermittently severe pyoderma gangrenosum without identifiable associated systemic disease. Her condition had become unresponsive to corticosteroid and sulfone therapy given for systemic effect, but responded completely to 150 mg/day of cyclophosphamide. Immunosuppressive therapy should be considered in patients with severe, recalcitrant pyoderma gangrenosum, even in the absence of associated systemic disease.

Adult↗