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Surgical management of pyoderma gangrenosum: case report and review.

BACKGROUND: Commonly used treatments for pyoderma gangrenosum are medical, with immunosuppressive agents employed most often. OBJECTIVE: To report a case and discuss the indications for radical surgical treatment of pyoderma gangrenosum. METHODS: Analysis of a case of Crohn's disease-associated pyoderma gangrenosum treated with immunosuppression followed by amputation, and a review of the literature on surgical management of pyoderma gangrenosum. RESULTS: In unstable patients with intractable multiple medical problems, surgical treatment of pyoderma gangrenosum may be indicated by the existence of these life-threatening comorbidities. The recent literature suggests that surgical management of pyoderma gangrenosum may also be appropriate in other special circumstances. CONCLUSIONS: Surgical management, including amputation, may have a role in the management of pyoderma gangrenosum. Further research is needed to delineate precisely the circumstances and patient factors that are appropriate indications for such surgery.

Aged↗

Pyoderma gangrenosum and ulcerative colitis.

The relationship of pyoderma gangrenosum and ulcerative colitis remains uncertain. We investigating 14 patients with pyoderma gangrenosum by colonoscopy with multiple biopsies. Six patients had ulcerative colitis and all of these had disease affecting the whole colon. There were no correlations between exacerbations of the colitis and the onset or course of pyoderma gangrenosum. The remaining eight patients with pyoderma gangrenosum had no other disease and they were found to be significantly older than those patients with coexisting colitis (P less than 0.002).

Adult↗

Pyoderma gangrenosum associated with biphenotypic acute leukemia.

Pyoderma gangrenosum is a neutrophilic dermatosis that may be associated with myeloid malignancies. Less information is available about the association of pyoderma gangrenosum with lymphoid malignancies. We present, to our knowledge, the first case of pyoderma gangrenosum associated with biphenotypic acute leukemia wherein the malignant cells show a phenotype specific for myelogenic and lymphocytic leukemia. Histopathologic examination revealed rather nonspecific features without involvement of leukemic cells in the skin lesions. Treatment with systemic steroids was followed by characteristically rapid healing of the skin lesion.

Female↗

[Pyoderma gangrenosum: successful topical therapy with tacrolimus (FK506)].

Pyoderma gangrenosum is a distinct clinical entity characterized by chronic, recurring, destructive ulcerations. Although the pathogenesis of pyoderma gangrenosum is unknown, immunologic aberrations of neutrophil granulocytes seem to be important. Systemic steroids and macrolide lactones such as cyclosporin A and tacrolimus have been reported to be useful in the clinical management of disease. Pyoderma gangrenosum has been found to be associated with several systemic diseases. The association with chronic ulcerative colitis is well known, but the diagnosis may be complicated by early administration of systemic steroids. Therefore, local immunosuppression with topically applied agents could be an efficient therapeutic alternative especially for mild or early cutaneous lesions.We describe the successful topical treatment of a patient with multiple lesions of pyoderma gangrenosum with 0,1% tacrolimus (FK506) ointment which is known to have better dermal penetration and higher immunosuppressive potency than topical cyclosporin A. In addition, other indications for topical tacrolimus are discussed.

Abdomen↗

Acute pyoderma gangrenosum does not require surgical therapy.

Pyoderma gangrenosum is an uncommon ulcerative skin disorder that is often associated with underlying systemic diseases, the most common of which is inflammatory bowel disease. We report an illustrative case of a 36-year-old woman with pyoderma gangrenosum occurring at surgical sites and at sites of trauma. She had associated Crohn disease. Multiple surgical procedures were performed on this patient, without improvement. Pyoderma gangrenosum can mimic a necrotizing soft tissue infection. Early recognition of the characteristic lesion may prevent unnecessary operations and facilitate effective control with appropriate medical therapy.

Acute Disease↗

Intravenous cyclosporine in refractory pyoderma gangrenosum complicating inflammatory bowel disease.

BACKGROUND: Pyoderma gangrenosum complicates inflammatory bowel disease in 2-3% of patients and often fails to respond to antibiotics, steroids, surgical debridement or even colectomy. METHODS: We performed a retrospective chart analysis of 11 consecutive steroid-refractory pyoderma patients (5 ulcerative colitis, 6 Crohn's disease) referred to our practice and then treated with intravenous cyclosporine. Pyoderma gangrenosum was present on the extremities in 10 patients, the face in 2, and stomas in 21. At initiation of intravenous cyclosporine, bowel activity was moderate in 3 patients, mild in 4, and inactive in 4. All patients received intravenous cyclosporine at a dose of 4 mg/kg/d for 7-22 days. They were discharged on oral cyclosporine at a dose of 4-7 mg/kg/d. RESULTS: All 11 patients had closure of their pyoderma with a mean time to response of 4.5 days and a mean time to closure of 1.4 months. All seven patients with bowel activity went into remission. Nine patients were able to discontinue steroids, and nine were maintained on 6-mercaptopurine or azathioprine. One patient who could not tolerate 6-mercaptopurine had a recurrence of pyoderma. No patient experienced significant toxicity. CONCLUSION: Intravenous cyclosporine is the treatment of choice for pyoderma gangrenosum refractory to steroids and 6-mercaptopurine should be used as maintenance therapy.

Adrenal Cortex Hormones↗

Pyoderma gangrenosum. A possible cause of wound necrosis.

Pyoderma gangrenosum is a destructive cutaneous disorder characterized by painful, rapidly enlarging ulcers with under-mined bluish and purplish red margins. This disease is most frequently found in association with ulcerative colitis. However, in the last three years, four cases of pyoderma gangrenosum precipitated by surgical procedures have occurred, and only one of these patients had ulcerative colitis. All four patients responded to steroid therapy and local wound care. Pyoderma gangrenosum can first be suspected by recognition of the previously described classic lesion. In addition, the absence of significant microbial growth and the lack of a specific etiology ascertained on routine and special staining of biopsy material is suggestive of the diagnosis. Histologic characteristics are entirely nonspecific but can be helpful in ruling out other specific entities. The progressively destructive nature of this process in the face of what would seem to be appropriate therapy for bacterial wound infection requires that pyoderma gangrenosum, as well as other aspects of the differential diagnosis of a persistent ulcerative lesion, such as fungal infection, factitious ulcer, or vasculitis, be considered.

Adult↗

Atypical bullous pyoderma gangrenosum associated with myeloid malignancies.

Atypical bullous pyoderma gangrenosum was diagnosed during the course of a myeloid malignancy in three patients. One patient had chronic myeloid leukaemia, one acute myeloid leukaemia, and the third, refractory anaemia with excess of blasts. This atypical form of pyoderma gangrenosum has been specifically associated with myeloid malignancies. The atypical appearance of the skin lesions and the clinical context in which they arose caused the true diagnosis to be delayed in all cases. Treatment with steroids was associated with rapid healing of the skin lesion. The histopathological changes in the skin biopsy specimens from these cases were non-specific, and although the histology was considered to be atypical of pyoderma gangrenosum in one case, the unusual features could be attributed to the patient's neutropenia. (Skin biopsy was performed to exclude other specific pathology). Atypical bullous pyoderma gangrenosum is an uncommon association of the myeloid malignancies. It may remain unrecognised and should be considered more often.

Adult↗

Pyoderma gangrenosum and cranial osteolysis: case report and review of the paediatric literature.

Pyoderma gangrenosum is a poorly understood, ulcerating cutaneous disorder which is rarely seen in the paediatric age-group. We report a 3-year-old boy who developed an ulcer over the left frontoparietal scalp at the age of 1 year. A 9-cm area of underlying cranial bone was destroyed. The appearance on radiographs and CT scan was suggestive of eosinophilic granuloma, osteomyelitis, or other destructive processes. Biopsies of the scalp lesion and calvaria showed granulation tissue and degenerating bone. After the biopsies the scalp lesion increased in size, and wound dehiscence occurred. Ulceration developed at the site of a PPD skin test, which on biopsy was consistent with the diagnosis of pyoderma gangrenosum. Pyoderma gangrenosum should be added to the differential diagnosis of cutaneous disorders which can result in osteolytic/osteonecrotic defects.

Humans↗

[Pyoderma gangrenosum: Clofazimine therapy].

Two patients with pyoderma gangrenosum have responded remarkably well to treatment with Clofazimine (Lamprène). The first patient, a 68-year old women suffered from pyoderma gangrenosum of the buttock and left leg and on the incision scar for cancer of the breast. Laboratory findings showed monoclonal dysglobulinemia (alpha 2-kappa 2). A daily dose of 300 mg of Clofazimine resulted in complete healing with ten days. The second patient was a 24-year old women suffering from ulcerative colitis and a rapidly progressing pyoderma gangrenosum of the left leg. The lesions was completely healed after two weeks of Clofazimine therapy. The dosage was 200 mg daily and was increased to 400 mg daily. Our cases showed decreased cellular immunity and their phagocytic activity was variable.

Adult↗

Histopathologic and immunopathologic study of pyoderma gangrenosum.

Sixty-three patients with pyoderma gangrenosum were seen and studied at the Mayo Clinic from 1971 to 1980. Biopsies from the erythematous border or necrotic edge of the pyoderma gangrenosum lesions usually demonstrated a characteristic pathogenic morphologic evolution. The early lesions revealed mild to moderate perivascular lymphocytic infiltrate associated with endothelial swelling. The fully developed lesions demonstrated necrosis in addition to a dense lymphocytic infiltration surrounding as well as involving the blood vessels. Extravasation of erythrocytes and thrombosis sometimes were seen. Ulceration, infarction, and abscess formation were found in the later stages of evolution. Direct immunofluorescence results were positive in the blood vessels of 36 of 65 (55%) specimens. IgM, C3, and fibrin were found in the papillary and reticular dermal vessels. IgG and IgA were only occasionally present. Pyoderma gangrenosum appears to be a reactive process that is manifested as a vasculitis. Biopsy material from the advancing active erythematous border has early characteristic dermatopathologic findings of lymphocytic vasculitis. Cutaneous vascular immune deposits suggest an immune pathogenesis of either an immune complex disease or lymphocytotoxic reaction.

Biopsy↗

Pyoderma gangrenosum associated with cystic acne and hidradenitis suppurativa controlled by adding minocycline and sulfasalazine to the treatment regimen.

Pyoderma gangrenosum often presents a difficult therapeutic challenge. The case is described of a 42-year-old black man with the association of cystic acne, hidradenitis suppurativa, and seronegative arthritis with pyoderma gangrenosum. The pyoderma gangrenosum ulcers remained refractory to treatment until therapies aimed in part at the associated diseases were begun. Minocycline was given for treatment of cystic acne and hidradenitis suppurativa as well as pyoderma gangrenosum. Sulfasalazine was prescribed for seronegative arthritis as well as pyoderma gangrenosum. The combination therapy permitted healing of the pyoderma gangrenosum ulcers.

Acne Vulgaris↗

Pyoderma gangrenosum in myelodysplasia and acute leukaemia.

Pyoderma gangrenosum is a rare occurrence in patients with haematological malignancy. This characteristic but nonspecific inflammatory process with skin destruction occurred in 4 patients with myelodysplasia, in one with acute leukaemic transformation of myelofibrosis, and in de novo acute myeloblastic leukaemia in another. Clinically, the cutaneous lesion in these patients differed from that associated with inflammatory bowel disease, arthritis, or the idiopathic type of pyoderma gangrenosum by having the vesiculo-bullous borders. Histopathological differences were also evident since more superficial layers of the skin were involved in the ulceration than typically encountered in patients with non-malignant systemic disease. Despite the less penetrating nature of this variant, treatment of the pyoderma gangrenosum is unsatisfactory and in the absence of effective therapy for the underlying disease, healing occurred only in the patient with acute leukaemia who achieved complete remission in response to chemotherapy.

Acute Disease↗

Pyoderma gangrenosum: classification and management.

Pyoderma gangrenosum (PG) has four distinctive clinical and histologic variants. Some have morphologic and histologic overlapping features with other reactive neutrophilic skin conditions. PG often occurs in association with a systemic disease, and the specific clinical features of the skin lesion may provide a clue to the associated disease. Management of PG depends on its type and severity and usually requires aggressive local and systemic treatment.

Humans↗

Pyoderma gangrenosum of the orbit.

Pyoderma gangrenosum is an uncommon chronic ulcerative condition, the aetiology of which is poorly understood. Ophthalmic involvement is rare. The case presented in this paper involves destruction of the orbital contents with subsequent perforation of the eye despite conventional therapy. Evisceration was performed with the patient being subjected to hyperbaric oxygen therapy pre- and post-operatively, with resultant cessation of the disease process.

Aged↗

Scrotal pyoderma gangrenosum associated with dermatomyositis.

Pyoderma gangrenosum (PG) of the scrotum is extremely rare. We report a case of scrotal PG associated with dermatomyositis. Initial healing was slow despite the use of oral prednisolone and azathioprine. Healing was accelerated by the local use of a potent topical corticosteroid. PG should be considered in the differential diagnosis of ulcerating lesions of the genitalia.

Administration, Topical↗

Pyoderma gangrenosum on the finger.

A case of pyoderma gangrenosum on the dorsal aspect of the right second and third fingers of a 50-year-old woman is reported. The patient had been wounded at the above-mentioned site by a cutter. This wound developed into a painful ulcer which enlarged rapidly. No bacterial infections were detected. Histologically, an inflammatory infiltrate of lymphocytes mixed with neutrophils and nucleodusts was seen throughout the entire dermis, but there was no evidence of vasculitis. Based on this data, the case was diagnosed as pyoderma gangrenosum. The patient responded to treatment with systemic corticosteroids. Pyoderma gangrenosum at this site of the fingers seems to be rare.

Female↗

Pyoderma gangrenosum complicating bilateral mammaplasty.

Pyoderma gangrenosum (PG) is an idiopathic necrotising cutaneous disorder. It is associated with systemic diseases like inflammatory bowel disease, monoclonal gammopathy, arthritides and haematological malignancy. PG occurring at sites of trauma, a phenomenon called pathergy, is well described. One of the manifestations of pathergy is PG occurring at sites of surgery. We describe a case of PG at sites of reduction mammaplasty and review the literature so far.

Adult↗