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Neutrophilic dermatoses: pyoderma gangrenosum and Sweet's syndrome.

Pyoderma gangrenosum and Sweet's syndrome are classified as neutrophilic dermatoses as they exhibit intense dermal inflammatory infiltrates composed of neutrophils with little evidence of a primary vasculitis. They share several characteristics and respond to immunosuppressives. Aetiology is felt to represent a manifestation of altered immunologic reactivity. Patients with both conditions concurrently have been described. Diagnosis is based on clinical and histopathological findings. However, clinically the typical forms of the two conditions are quite distinct: pyoderma showing cutaneous ulceration with a purple undermined border and Sweet's syndrome having tender, erythematous, nonulcerated plaques and nodules. Approximately 50% of cases of pyoderma are associated with a specific systemic disorder. These include inflammatory bowel disease, rheumatoid arthritis, non-Hodgkin's lymphoma and myeloproliferative disorders. Many associations with Sweet's syndrome have been described, including acute myeloid leukaemia, myeloma and adenocarcinomas, and haematological malignancy. There is overlap between the two conditions with lesions categorised as Sweet's syndrome being clinically more characteristic of atypical pyoderma and vice versa. We believe that pyoderma and Sweet's syndrome represent a continuum of spectrum of disease. The reason for the clinical differences between the conditions is unclear and merits further investigation but may be explained by varying levels of intensity and extent of the inflammatory process. This review will describe the pathogenesis, clinical features, diagnosis, associations and treatment of the two conditions.

Aged↗

Pyoderma gangrenosum complicating hysterectomy for fibroids.

Pyoderma gangrenosum is a destructive, non-infective ulceration of the skin. The case presented illustrates a rare but important presentation in a patient following a total abdominal hysterectomy and bilaterial salpingo-oophorectomy (TAH and BSO).

Female↗

Polyarthritis with perinuclear antineutrophil cytoplasmic antibody and Pyoderma gangrenosum. Report of a case.

The authors report a case of Pyoderma gangrenosum with seronegative nondestructive polyarthritis and perinuclear antineutrophil cyoplasm antibody. Rheumatic disorders that occur in association with Pyoderma gangrenosum are reviewed. The constellation of manifestations in the authors' patient does not have been reported previously and may represent a new entity among the rheumatic manifestations of Pyoderma gangrenosum.

Adult↗

Familial pyoderma gangrenosum presenting in infancy.

UNLABELLED: Pyoderma gangrenosum (PG) is a rare, poorly understood skin disease that occurs in all age groups. Less than 0.4% of patients are infants and represent a diagnostic challenge as early lesions may resemble other skin disorders. Here we report for the first time three siblings affected with PG all presenting during infancy. Unlike the older age group, the ulcers spared the legs but involved the buttocks, thighs and perianal area in all the infants. CONCLUSION: This is the first reported family with PG affecting three siblings suggesting autosomal recessive inheritance. The diagnosis may be more difficult in infants due to absence of underlying associated disorders and the tendency of the lesions to appear in areas where infants frequently have other dermatoses. PG characteristically involves the buttocks, thighs and perianal area and spares the legs.

Adolescent↗

Peristomal pyoderma gangrenosum and inflammatory bowel disease.

Pyoderma gangrenosum (PG) is a debilitating skin disease most often associated with inflammatory bowel disease and is a reportedly rare cause of peristomal ulceration. The lesions of PG rapidly evolve from small, erythematous pustules to deep, painful, pyogenic ulcers within hours to days of onset. Although the behavior and the appearance of the lesions of peristomal PG are diagnostic, a lack of familiarity with PG often leads to misdiagnosis and inappropriate therapy. This study reports four cases of peristomal PG and discusses the 20 previously reported cases in patients with inflammatory bowel disease. Seventy-five percent of patients were female and 67% had Crohn's disease. All patients had colitis, including all of the patients with Crohn's disease, 82% of whom had additional perineal complications. The diagnosis of peristomal PG was based on clinical appearance alone in 83% of cases. The onset of peristomal PG ranged from 2 weeks to 3 years following ostomy. The response to medical therapy was variable. All cases (17 of 17) treated with high-dose corticosteroids and local wound care responded, but five cases required additional therapy. No patient was successfully treated with stoma revision. Risk factors for the development of peristomal PG include Crohn's colitis, female gender, and perineal disease. While most patients respond well to systemic steroids and local wound care, up to one third of patients require long-term medical management.

Adolescent↗

Intravenous cyclophosphamide pulses in pyoderma gangrenosum: an open trial.

OBJECTIVE: To evaluate the potential efficacy of intravenous bolus cyclophosphamide (IVCY) in patients with pyoderma gangrenosum. METHODS: Consecutive patients with a diagnosis of pyoderma gangrenosum seen in a period of 3 years in tertiary care referral center were included. Patients received IVCY 500 mg/m2 of body surface area, every month until reaching a maximum of 6 doses, or healing of their ulcers or a lack of response after 3 doses. Patients were assessed every month during the time they received IVCY and every 3 months thereafter. The assessments included number and size of ulcers, and a safety profile of the study drug. Complete remission was defined as 100% ulcer healing, partial remission as a decrease > or = 50% but less than 100%, and therapeutical failure if the size of the ulcer increased or decreased < 50%. RESULTS: Nine patients were included, 6 were men, the mean age was 46 yrs (range 24-76). The mean disease duration was 3.3 yrs (range 1 week to 9 yrs). Four patients had idiopathic pyoderma gangrenosum, 3 had associated rheumatoid arthritis, and 2 had associated systemic lupus erythematosus. Complete remission was observed in 7 patients, partial in one, and failure in one. Relapses were observed, 3 months after the last IVCY (2 cases) and 12 months after the last IVCY (one case). Transitory thrombocytopenia and leukopenia developed in one patient and nausea and vomiting in another. CONCLUSION: IVCY appears effective in controlling the lesions of pyoderma gangrenosum and inducing remission for a substantial period in many individuals.

Adult↗

A case of pyoderma gangrenosum stabilized with lymecycline, topical benzoyl peroxide and treated by autograft.

Pyoderma gangrenosum is a chronic inflammatory ulcerative skin disease of unknown etiology, often associated with various systemic disorders such as inflammatory bowel disease, rheumatoid arthritis, chronic active hepatitis, diabetes mellitus and hematologic malignancies. The ulcers are characterized by their undermined violaceous borders. The disease remains a therapeutic challenge. Corticosteroids are the mainstay of therapy; however, side effects from this treatment and recalcitrant pyoderma gangrenosum require therapeutic alternatives. We report the case of a large subacute pyoderma gangrenosum stabilized with lymecycline, topical benzoyl peroxide and successfully treated by an autograft. This observation supports the opinion that the risk of pathergy of a graft can be avoided by the stabilization of the disease.

Acute Disease↗

Present status of pyoderma gangrenosum. Review of 21 cases.

This article summarizes the management of 22 cases of pyoderma gangrenosum over the past four years at the hospital of the University of Pennsylvania, Philadelphia. Eighteen patients with pyoderma gangrenosum were studied using the most sensitive routine laboratory method for detection of monoclonal immunoglobulins, immunofixation electrophoresis. Four cases of IgA gammopathy were detected, confirming previous reports of the incidence of monoclonal gammopathy in pyoderma gangrenosum. High-dose glucocorticoid therapy (pulse therapy) is an effective treatment for some severe, refractory cases of pyoderma gangrenosum. Eight patients were treated with pulse therapy. Six responded favorably, and none had serious complications.

Adrenal Cortex Hormones↗

[Pyoderma gangrenosum after TAPP hernioplasty. A rare differential necrotizing wound infection diagnosis].

Pyoderma gangrenosum is an aseptic skin disease that occasionally complicates operative incisions and mimics postoperative necrotising wound infection. So far there are only a few case reports about bacterial necrotising infections following laparoscopy; no report exists about postoperative pyoderma gangrenosum after minimally invasive surgery. Differential diagnosis of both these diseases with potentially high morbidity and mortality is, however, essential, as they require opposite therapeutic regimens. Here we present the case of a patient who developed pyoderma gangrenosum after laparoscopic hernioplasty. Pathophysiological, clinical and therapeutic aspects of the disease are discussed.

Aged↗

[Pyoderma gangrenosum--possible diagnosis in therapy-resistant wounds].

Pyoderma gangrenosum is a chronic ulcerative inflammatory skin disease. The condition is often associated with inflammatory bowel disease. We report three patients with pyoderma gangrenosum successfully treated with cyclosporin. There were difficulties in establishing the diagnosis, and antibiotics, surgical excisions and different wound dressings had been used without effect.

Adolescent↗

Pyoderma gangrenosum with large circumferential perianal skin loss in a child.

Pyoderma gangrenosum is an uncommon skin disorder characterised by deep ulcers surrounded by a violaceous over-hanging edge. Although in many instances there is no clear association with any underlying disease, pyoderma gangrenosum has been described in ulcerative colitis, Crohn's disease, polyarthritis, diabetes mellitus and myeloma. Pyoderma gangrenosum may also be seen as a rare manifestation of myeloproliferative disease including leukaemia. In children, as in our case, it may be the presenting feature.

Anal Canal↗

Pyoderma gangrenosum and monoclonal gammopathy.

The records of eight patients with pyoderma gangrenosum and monoclonal gammopathy showed that all patients except one had an IgA paraproteinemia. To date, seven patients have had a benign course and multiple myeloma has developed in one. In seven patients, the onset of the pyoderma gangrenosum preceded the detection of the monoclonal gammopathy. The monoclonal gammopathy did not seem to influence the morphologic findings, course, or therapy of the pyoderma gangrenosum. In the one patient with myeloma, treatment of the myeloma caused accelerated healing of the skin lesions.

Adult↗

[Clinical analysis of 6 cases of pyoderma gangrenosum].

This article reports 6 cases of pyoderma gangrenosum of either acute or chronic clinical type. Etiologically, we found that minor local trauma could induce ulcerative and destructive lesions, with typical pyoderma gangrenosum features following in 4 cases. All the patients responded well to systemic corticosteroids. In comparison with cases reported in other countries, no systemic diseases (including ulcerative colitis, Crohn's disease and polyarthritis) were found in our series. Cultures taken from early pustular lesions were sterile. Histopathological examination showed heavy neutrophilic infiltration in the dermis, and no evidence of vasculitis was found in the biopsies. Immunological investigations revealed no specific reactions in our cases.

Adolescent↗

Multiple pulmonary nodules in association with pyoderma gangrenosum.

This report describes a patient with extensive pyoderma gangrenosum in whom there were co-existent lung abnormalities. The patient's X-ray showed peripherally sited multiple pulmonary lesions bilaterally. A lung biopsy showed chronic non-specific inflammatory changes with neutrophil and lymphocyte infiltration which were similar to the skin lesions. This case was diagnosed as multiple aseptic nodules in pyoderma gangrenosum. The pulmonary infiltrative shadows were controlled only with prednisolone treatment. Steroid therapy is considered to be the first choice to control pulmonary lesions of this disease.

Anti-Inflammatory Agents↗

Accelerated healing of pyoderma gangrenosum treated with bioengineered skin and concomitant immunosuppression.

Pyoderma gangrenosum is a rare, destructive, neutrophilic dermatosis, the origin of which remains largely obscure. The ulcerative variant of this inflammatory disorder causes painful, necrotic, rapidly enlarging ulcers. Because of pathergy, many clinicians avoid managing these nonhealing ulcers with aggressive surgical debridement and autologous grafts. This article proposes that the application of an allogeneic cultured human skin equivalent (Graftskin) not only circumvents this problem, but also hastens re-epithelialization of the ulcer bed. An added benefit of the possible improvement of the cosmetic appearance of the final scar by preventing severe wound contracture is also postulated. We report a newly diagnosed case of ulcerative pyoderma gangrenosum; the use of bioengineered skin as an adjunct to concurrent immunosuppressive therapy with cyclosporine hastened the healing and diminished pain in a rapidly enlarging leg ulcer. Within 2 weeks, the ulcer was 30% to 40% healed, achieving 100% re-epithelialization within 6 weeks.

Adult↗

Malignant pyoderma or pyoderma gangrenosum of the head and neck?

Malignant pyoderma is a rapidly progressive ulcerating process of unknown origin that predominantly affects the head and neck of young adults. Malignant pyoderma has been considered distinct from pyoderma gangrenosum because of the predominant head and neck location of the ulcers and because the ulcers lack undermining and surrounding erythema. A case of a 22-year-old woman with a severe ulcerative process predominantly affecting the head and neck is described. A review of the literature indicates that malignant pyoderma and pyoderma gangrenosum are almost certainly identical disorders.

Adult↗

Peripheral ulcerative keratitis--an extracutaneous neutrophilic disorder: report of a patient with rheumatoid arthritis, pustular vasculitis, pyoderma gangrenosum, and Sweet's syndrome with an excellent response to cyclosporine therapy.

The term peripheral ulcerative keratitis represents a spectrum of inflammatory diseases, characterized by cellular infiltration, corneal thinning, and ulceration. Neutrophilic dermatoses are rarely associated with peripheral ulcerative keratitis. To date, peripheral ulcerative keratitis has only been reported in patients with pyoderma gangrenosum. Separate episodes of pyoderma gangrenosum, Sweet's syndrome, and pustular vasculitis developed in a 60-year-old patient with rheumatoid arthritis over an 8-year period. Over the past 2 years, 3 episodes of peripheral ulcerative keratitis occurred. Cyclosporine (4 mg/kg/d) treatment was started on confirmation of pyoderma gangrenosum. Over the ensuing 2 years, it became evident that the activity of her ocular and skin diseases, as well as her arthritis, paralleled the administration or cessation of cyclosporine therapy. Dermatologists should be aware of the association of Sweet's syndrome, pyoderma gangrenosum, and pustular vasculitis with peripheral ulcerative keratitis. This rare ocular manifestation and the serious sequelae when left untreated make recognition crucial. Cyclosporine proved to be a very effective treatment for all of our patient's diseases.

Arthritis, Rheumatoid↗