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Clinical management of pyoderma gangrenosum.

Pyoderma gangrenosum is a noninfectious neutrophilic dermatosis that usually starts with sterile pustules which rapidly progress to painful ulcers of variable depth and size with undermined violaceous borders. In 17 to 74% of cases, pyoderma gangrenosum is associated with an underlying disease, most commonly inflammatory bowel disease, rheumatological or hematological disease or malignancy. Diagnosis of pyoderma gangrenosum is based on a history of an underlying disease, typical clinical presentation and histopathology, and exclusion of other diseases that would lead to a similar appearance. Randomized, double-blinded prospective multicenter trials investigating the treatment of pyoderma gangrenosum are not available. The treatments with the best clinical evidence are systemic corticosteroids (in the initial phase usually 100 to 200 mg/day) and cyclosporine (mainly as a maintenance treatment). Combinations of corticosteroids with cytotoxic drugs such as azathioprine, cyclophosphamide or chlorambucil are used in patients with disease that is resistant to corticosteroids. The combination of corticosteroids with sulfa drugs, such as dapsone, or clofazimine, minocycline and thalidomide, has been used as a corticosteroid-sparing alternative. Limited experience has been documented with methotrexate, colchicine, nicotine, and mycophenolate mofetil, among other drugs. Alternative treatments include local application of granulocyte-macrophage colony-stimulating factor, intravenous immunoglobulins and plasmapheresis. Skin transplants (split-skin grafts or autologous keratinocyte grafts) and the application of bioengineered skin is useful in selected cases in conjunction with immunosuppression. Topical therapy with modern wound dressings is useful to minimize pain and the high risk of secondary infection. The application of topical antibacterials cannot be recommended because of their potential to sensitize and their questionable efficacy, but systemic antibacterial therapy is mandatory when infection is present. Despite recent advances in therapy, the prognosis of pyoderma gangrenosum remains unpredictable.

Adrenal Cortex Hormones↗

Simultaneous bilateral microvascular tissue transfer for soft-tissue defects of the lower extremities in pyoderma gangrenosum.

Pyoderma gangrenosum is a necrotizing skin disease of unknown etiology. The lesions are painful and occur mostly on the lower extremities, either single or multiple, but can appear in any region of the body. The authors report a case of pyoderma gangrenosum involving bilateral skin lesions over the anterior surface of the legs in a 13-year-old male patient. The lesions presented with ulcerative colitis, which is one of the most commonly associated diseases of pyoderma gangrenosum. After examination, systemic immunosuppressive treatment was administered and the progression of the disease was controlled. The lesions were treated with the simultaneous application of two free anterolateral thigh fasciocutaneous flaps after radical debridement. The procedure was successfully performed and no recurrence was observed. Although microvascular free tissue transfer cannot be performed in a standard fashion in pyoderma gangrenosum cases because of the risk of pathergy response, it should be considered as a surgical option in selected cases, otherwise difficult to manage, with the simultaneous application of appropriate systemic medical treatment.

Adolescent↗

Caring for the patient with peristomal pyoderma gangrenosum.

Pyoderma gangrenosum is an ulcerating skin condition associated with inflammatory bowel disease and other diseases. During and 18-month period beginning in the fall of 1991, seven patients were followed up for pyoderma gangrenosum. Three of these cases, with assessment and treatment plans, are presented. A discussion of treatment principles for managing pyoderma gangrenosum follows.

Adult↗

Bullous pyoderma gangrenosum.

Pyoderma gangrenosum is a necrotizing, ulcerative process commonly associated with inflammatory bowel disease but also occurring in several other systemic illnesses. When associated with myelodysplastic syndromes, its clinical appearance is often atypical. We describe a patient with severe, refractory bullous pyoderma gangrenosum. At autopsy cardiac involvement was demonstrated, a finding not previously reported. Patients with this unusual variant of pyoderma gangrenosum have a grave prognosis.

Female↗

Case study: diagnosis and treatment of pyoderma gangrenosum.

Pyoderma gangrenosum, is an ulcerative, inflammatory disorder often associated in the field of stoma care with inflammatory bowel disease (IBD) (Lyon and Smith, 2001). Pyoderma gangrenosum is distinctive in that it normally has a purple rolled edge and is extremely painful. The pathogenesis is unknown but immunologic aberrations of neutrophil granulocytes seem to be important (Petering et al, 2001). This paper demonstrates the rapid development and effective multi-disciplinary team management of an extensive case of Pyoderma gangrenosum. It also explores the possible causes of this particular manifestation.

Colostomy↗

[Ulceration of the scalp: a unique manifestation of pyoderma gangrenosum].

Pyoderma gangrenosum is a chronic, noninfectious, ulcerating cutaneous disease of unknown etiology usually presenting with multiple lesions on the legs and trunk. Solitary lesions in other locations are rare and therefore hard to diagnose. A 78-year-old and a 81-year-old woman with extensive scalp necrosis were both diagnosed as pyoderma gangrenosum after exclusion of other differential diagnostic possibilities. The separate diagnosis of malignant pyoderma for this disease state no longer seems justified. Scalp necrosis is infrequently seen in dermatological patients; most previous reports describe it in giant cell arteritis. Other differential diagnoses include viral, bacterial and mycotic infections as well as erosive pustular dermatosis of the scalp. In conclusion, we again draw attention to unilesional pyoderma gangrenosum of the scalp as a rare disease entity. Immunosuppressive drugs are an effective treatment modality.

Aged↗

Favorable response to infliximab treatment in a patient with active Crohn disease and pyoderma gangrenosum.

Pyoderma gangrenosum is an extraintestinal manifestation of inflammatory bowel disease requiring meticulous medical and/or surgical treatment. We describe a 46-year-old patient who developed harsh pyoderma gangrenosum during a severe flare-up of the underlying Crohn disease of the terminal ileum. The patient responded favorably to treatment with infliximab-the chimeric antibody against tumor necrosis factor-alpha. The drug was administered intravenously at a dose 5 mg/kg/BW at baseline and weeks 2 and 6. Abdominal signs and symptoms as well as the skin lesions improved markedly before the second infusion. The patient is presently on infliximab maintenance regimen at a dose of 5 mg/kg/BW being administered as a 3 dose loading regimen at 0, 2 and 6 weeks with a treatment-free interval of 10 weeks until the next loading dose. The skin lesions remained in remission. Infliximab is a promising therapeutic modality for patients with Crohn disease and pyoderma gangrenosum.

Antibodies, Monoclonal↗

A case study of pyoderma gangrenosum.

Pyoderma gangrenosum is a rare neutrophilic dermatosis seen initially as painful pustules or bullae on the skin that rapidly ulcerate and have a characteristic raised, purplish areola surrounding them. It is often associated with inflammatory bowel disease and less commonly with other systemic diseases, such as myelofibrosis, rheumatoid arthritis, and chronic active hepatitis. Pyoderma gangrenosum involving the hand is a rare entity, with only two previous cases reported in the literature. This article describes a patient with myelofibrosis in whom pyoderma gangrenosum of the hand developed after she underwent splenectomy.

Aged↗

Vulvar pyoderma gangrenosum.

Pyoderma gangrenosum is an idiopathic dermatologic disease manifested by painful cutaneous ulceration. The ulcers are characterized by their undermined, violaceous borders and necrotic tissue at the ulcer base. The lesions may have an unusual response to physical manipulation known as pathergy, a phenomenon that is manifested by rapid progression following debridement. Pyoderma gangrenosum is frequently associated with systemic diseases such as inflammatory bowel disease, rheumatoid arthritis, chronic active hepatitis and hematologic malignancies. Conservative wound care and systemic corticosteroids are usually effective therapy. We report the second case in the gynecologic literature of a patient with vulvar pyoderma gangrenosum.

Adult↗

Pituitary granuloma and pyoderma gangrenosum.

Pyoderma gangrenosum is a rare chronic and recurrent skin disease characterized by progressing lesions from papulopustules to large necrotic sterile ulcers. Its definite etiology remains unknown. In a 40-year-old woman with typical pyoderma gangrenosum an intrasellar mass with suprasellar extension was diagnosed and removed by transsphenoidal surgery. Histopathological features of the lesion were those of a nonspecific granulomatous hypophysitis. Five months postoperatively the patient experienced visual defects and hypopituitarism demonstrated by endocrine evaluation. Computerized tomography showed the recurrence of the intrasellar expanding mass. Extensive and repeated evaluation failed to find any evidence of sarcoidosis, tuberculosis or histiocytosis. Corticosteroid therapy was preferred to surgery and 80 mg daily prednisone produced a dramatic shrinkage of the pituitary pseudotumor. Long-term follow-up studies did not disclose any recurrence of the pituitary granulomatous process nor objective evidence of underlying disease even after steroid dosage has been tapered. The hypothesis of a pituitary localization of pyoderma gangrenosum is suggested by the similarity between the histopathologic findings of the two conditions and the excellent response to steroid therapy.

Adult↗

Penile pyoderma gangrenosum.

Pyoderma gangrenosum is a rare ulcerating inflammatory skin disease. Genital involvement has been rarely reported. We report a 24-year-old man with penile pyoderma gangrenosum who was treated with systemic corticosteroids.

Adult↗

Successful surgical treatment of Takayasu's arteritis associated with pyoderma gangrenosum.

Pyoderma gangrenosum is rarely associated with Takayasu's arteritis. We report the successful surgical treatment of a 34-year-old woman who was diagnosed with pyoderma gangrenosum associated with thoracic aortic aneurysm and dissection due to Takayasu's arteritis. She underwent graft replacement of the thoracic aortic aneurysm under cardiopulmonary bypass, with perioperative management using prednisolone and cyclosporine A. She has had no evidence of the development of skin lesions or the progression of Takayasu's arteritis.

Adult↗

Clinical treatment options for peristomal pyoderma gangrenosum.

Pyoderma gangrenosum is a rare debilitating skin disease of unknown aetiology that manifests itself as painful lesions and is often associated with inflammatory bowel disease. Stoma care nurses will encounter it directly around the stoma as peristomal pyoderma gangrenosum (PPG). There are many case reports of varying methods of treatment for PPG, although none have proven to be efficacious in all circumstances. When these lesions develop in the peristomal area, treatment must be balanced with the need to maintain an intact stoma appliance.

Colitis, Ulcerative↗

Pyoderma gangrenosum.

Pyoderma gangrenosum is an uncommon skin disorder often associated with systemic disease, especially chronic ulcerative colitis (Figures 1, 2, and 3). Therapy is twofold--local care and control of the underlying illness. While the overall prognosis is good and the disease is rarely fatal, pyoderma gangrenosum is physically and mentally crippling with a propensity for recurrence.

Aged↗

Pyoderma gangrenosum.

Pyoderma gangrenosum is a clinical diagnosis. The ulcer shows three absolutely distinctive morphologic features: a purple-red, raised, undermined border; an irregular base, and cribriform scarring at the periphery. Evaluation of a patient with pyoderma gangrenosum requires a systematic search for possible associated inflammatory bowel disease or other internal disease. Although a variety of local treatments are used, systemic steroids are almost always necessary.

Adrenal Cortex Hormones↗

New look at pyoderma gangrenosum.

Pyoderma gangrenosum cannot be viewed simply as "an ulcer with undermined bluish borders," since this description recognizes only one stage of the evolving process. Patients with this disorder often have cutaneous lesions including papules, pustules, and plaques that evolve and resolve without ever passing through an ulcerative stage. Moreover, such patients frequently have a variety of internal inflammatory and ulcerative disorders. The nature of these disorders as well as the increasing evidence of grossly altered immunity in pyoderma gangrenosum is also presented.

Antigens, Bacterial↗

[Pyoderma gangrenosum].

Pyoderma gangrenosum is a very strange disease usually diagnosed on clinical grounds only and without any characteristic biological disturbance. It must be neither missed nor diagnosed too easily at the expense of vascular or infective ulcerations which are much more frequent. Its physiopathology remains shrouded in mystery since the abnormalities that have been found were extremely varied and sometimes conflicting, and were observed in short series. Thus, pyoderma gangrenosum appears as a syndrome which if often causeless or due to multiple diseases and might well be split into different entities in the forthcoming years. Despite its obscure pathogenesis, most patients can be cured by systemic corticosteroid therapy, sulfones or clofazimine. The main point of interest of that disease is that in almost 50 percent of the cases it is associated with a severe underlying pathology.

Adrenal Cortex Hormones↗