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Pyoderma gangrenosum (PG) is an ulcerative skin disorder of unknown aetiology. We describe a 40-year-old woman with long-standing PG and who did not respond to the usual treatments; however, she was treated successfully with plasmapheresis followed by pulse intravenous cyclophospamide therapy.
Pyoderma gangrenosum (PG) is a rare, ulcerative skin disease. Diseases with cutaneous manifestations resembling PG may lead to the misdiagnosis of PG. We discuss two patients presenting with cutaneous ulcers originally thought to be PG, and review the literature on the clinical and pathologic characteristics of PG ulcers compared to PG-like ulcers in order to determine possible distinguishing features.
Pyoderma Gangrenosum (PG) is often associated with an underlying disease. PG as a paraneoplastic disease is illustrated by the presentation of four patients with malignancy of myeloproliferative origin and PG. An associated malignancy is found in approximately 7% of patients with PG, most commonly haematologic in nature and in particular leukaemia. Clinically the PG is often of the superficial bullous variant and is associated with a poor prognosis.
Pyoderma gangrenosum (PG) is an idiopathic inflammatory disease of unknown aetiology, frequently associated with an underlying systemic condition such as inflammatory bowel disease or haematological malignancy. Its occurrence tends to parallel exacerbations of the underlying disease. Four clinical variants of PG have been described and these include ulcerative, pustular, bullous and vegetative types. We report two cases of the pustular form, which is an uncommon variant of PG, where the pustules do not progress to form ulcers. Both our patients suffered with inflammatory bowel disease which remained quiescent as the pustular PG developed.
Pyoderma gangrenosum (PG) is an idiopathic, painful and destructive condition that usually presents as an ulceration on the pretibial region of the legs. It primarily affects patients with inflammatory bowel disease, arthritis, myeloproliferative disorders and chronic hepatitis, and occasionally affects patients with other conditions, but it may also occur without any associated illness. Since there are no specific serologic or histologic markers for PG, it must be diagnosed clinically. Treatment usually involves immunosuppressant therapy as well as treatment of the underlying disease. The course of PG is unpredictable, and prognosis depends on the extent of the skin lesions at the time of diagnosis, underscoring the need for early and aggressive management.
Pyoderma gangrenosum (PG) is a type of neutrophilic disorder with a chronic clinical course. Immunosuppressive agents have been used for its management. Among them, corticosteroid is known as the most effective. However, other immunosuppressants including cyclosporine A have been selected for patients with PG who were refractory to systemic steroids. Herein we report a case of PG resistant to systemic steroids, who was successfully treated with topical tacrolimus. A fifty-four year-old male had a 14-year history of PG. In 2002, necrotic ulcers appeared on his right leg that were refractory to oral prednisolone (30 mg/day). The application of topical tacrolimus to the border of the ulcers hastened epithelization of the ulcers and allowed for reduction of the oral prednisolone. Topical tacrolimus therapy may be an effective alternative for PG when the lesion is poorly controlled by corticosteroid.
Pyoderma gangrenosum (PG) is a rare chronic inflammatory skin disease characterized by the recurring development of necrotizing and painful ulcers. The skin lesions appear spontaneously or after minor traumatic injuries. Sites of predilection include the lower limbs and the trunk but any part of the body may be affected. PG is not an infectious disease; although the etiology is not completely understood, an immune disturbance is certainly involved. An underlying systemic disorder is associated in up to 50% of the cases, specially inflammatory bowel diseases, arthritis, paraproteinemias and hematologic malignancies. Chronic venous or arterial ulcers as well as bacterial gangrene are the most frequent false diagnoses. A right diagnosis, based upon the distinctive clinical features and a compatible histology, is essential to avoid surgical procedure that often tends to exacerbed the process. Because of its persistent and recurrent nature, systemic long-term therapy based upon corticosteroids associated with sulfones or immunosuppressive agents is required.
Pyoderma gangrenosum (PG) is a non-infectious reactive neutrophilic dermatosis which typically starts with pustules which rapidly evolve to painful ulcers of variable size and depth with undermined violaceous borders. Since its first description in 1930, the pathogenesis of PG has remained elusive even as an ever-widening range of systemic diseases has been described in association with it. The diagnosis of PG is based on clinical and pathologic features and requires exclusion of other conditions that produce ulcerations, since misdiagnosis exposes patients to risks associated with treatment. Critical to proper management are correct diagnosis, identification and treatment of any underlying disorder, and the appropriate choice of topical and systemic therapy. PG has four distinctive clinical and histologic variants, and the specific clinical features of the lesion may provide a clue to the associated disease. The most common associated diseases are inflammatory bowel disease, rheumatological or hematological disease or malignancy. Although there is no single successful treatment for PG, certain type of PG lesions are recognized to respond more readily to accepted therapies than others. Local treatment may be sufficient for mild disease, while systemic immunosuppressive therapy is necessary for severe cases. The treatments with the best clinical evidence are oral or pulse intravenous corticosteroids, and cyclosporine. Surgical therapy is useful in selected cases in conjunction with immunosuppression. Wound stabilization is obtained only through control of the systemic and local inflammatory process. Emerging therapies include use of platelet-derived growth factor and cell culture grafts when re-epithelialization is slow, and the TNF-alpha blocking agent infliximab for refractory disease. Despite advances in therapy, the long-term outcome for patients with PG remains unpredictable, because relapses are common.
Pyoderma gangrenosum is a serious skin disorder. About half of the cases are associated with systemic diseases such as inflammatory bowel disease or arthritis. Many management regimens have been recommended, but treatment must be individualized and must include concern for the physical and psychologic needs of the patient. This particularly stressful and uncertain lesion is a challenge to the physician.
Pyoderma Gangrenosum is cutaneous manifestation of a systemic problem that should be systematically diagnosed and managed with care. When such lesions appear on legs, it is important to study and treat the systematic problem.
BACKGROUND: Pyoderma gangrenosum is a diagnosis of exclusion, and the misdiagnosis of pyoderma gangrenosum can result in substantial complications in patients who have other causes of severe cutaneous ulceration. METHODS: We reviewed the charts of 240 patients with a diagnosis of pyoderma gangrenosum who were evaluated at our institution from 1975 through 2000, including 157 consecutive patients treated for presumed pyoderma gangrenosum from 1984 through 1992. We also reviewed the English-language literature. RESULTS: Ninety-five patients (49 from our institution and 46 described in the literature) had skin ulcers with a clinical resemblance to pyoderma gangrenosum. The final diagnoses were vascular occlusive or venous disease, vasculitis, cancer, primary infection, drug-induced or exogenous tissue injury, and other inflammatory disorders. Of the 95 patients studied, 64 had been treated for pyoderma gangrenosum for a median of 10 months (range, 3 to 180). These 64 included 15 of the 157 consecutive patients treated for pyoderma gangrenosum at our institution (10 percent). Of the ulcers in the 64 patients treated for pyoderma gangrenosum, it was clear that those in 23 patients (36 percent) did not respond to treatment directed at pyoderma gangrenosum, those in 8 (12 percent) were exacerbated by such treatment, and those in 15 (23 percent) improved with such treatment. CONCLUSIONS: The misdiagnosis of pyoderma gangrenosum is not uncommon and exposes patients to risks associated with its treatment. A thorough evaluation is required in all patients suspected of having pyoderma gangrenosum in order to rule out alternative diagnoses.
A case simulating extraordinarily extensive chronic vegetating pyoderma revealed itself in its course to be pyoderma gangrenosum. The bacterial flora varied due to a secondary colonization; there was no response to antibiotics but there was to corticosteroid, azathioprine and clofazimine therapy. IgA-paraproteinemia was found. Pyoderma gangrenosum has historically been considered to be a chronic ulcerous pyoderma and is not a disease of bacterial origin. Reviewing the literature available since the turn of this century, chronic vegetating pyoderma is compared with the latter from the viewpoint of clinical features, histopathology, immunology, course and treatment. There are so many convincing parallels between the two conditions that we would like to propose that we stop regarding them as distinct entities and instead view them as parts of a spectrum of the same immunopathological process.
Vegetative pyoderma gangrenosum is a nonaggressive, chronic, superficial variant of pyoderma gangrenosum. Unlike classic ulcerative pyoderma gangrenosum, the lesions are less deep and not painful, and the borders are not undermined. The base of ulcers usually shows granulation tissue. The histopathologic findings are characterized by superficial granulomas. Cultures for fungal or bacterial infections normally are negative. Vegetative pyoderma gangrenosum is seldom associated with systemic diseases and often responds to simple modes of therapy. Our patient had a 10 years history of superficial ulcers on face, trunk and upper arms, whose general health was not impaired. A broad diagnostical evaluation showed no systemic diseases. The histologic pattern demonstrated a granulomatous dermatitis. After exclusion of other causes, we believe the patient has the vegetative form of pyoderma gangraenosum. There was no improvement with local treatment. Only systemic therapy with corticosteroids and later on cyclosporine led to healing.
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BACKGROUND: Pyoderma gangrenosum is an uncommon skin disease usually treated with systemic drugs, most frequently corticosteroids. Isolated topical treatment is usually considered unsatisfactory. OBJECTIVE: To evaluate the effect of topical 1% sodium cromoglycate solution on pyoderma gangrenosum in 5 patients 25-30 years of age. METHODS: 5 patients, including 2 under systemic steroid treatment, hospitalized in our department from 1992 to 1993 because of pyoderma gangrenosum, were treated with topical 1% sodium cromoglycate solution. Systemic corticosteroids were further added in 2 patients whose initial improvement was inadequate. RESULTS: Initial improvement was noted in all 5 patients after 3-7 days of sodium cromoglycate treatment. Complete healing of the ulcers occurred within 5-8 weeks. CONCLUSIONS: Topical treatment with sodium cromoglycate can be effective as adjunctive or sole treatment in pyoderma gangrenosum.
Five patients (four black Nigerian males and one Polynesian) with pyoderma gangrenosum (PG) were seen between May 1974 and March 1984, at the Ahmadu Bello University Teaching Hospital, Kaduna, Nigeria. Their age range was 12-42 years (mean 25.6 years). The female expatriate Polynesian patient had PG localized to the upper back while the other four patients had severe and extensive PG lesions. Local cleansing and dressing of the ulcers combined with appropriate systemic antibiotics produced healing in two of these patients. The empirical addition of dapsone and rifampicin led to complete healing in two others, but only transient remission in one patient who, after 22 years of disease activity, died at home from an aggressive and accelerated form of the disease best described as 'malignant pyoderma' gangrenosum.
Peristomal pyoderma gangrenosum (PPG) is a variant of pyoderma gangrenosum (PG) that is more refractory to treatment. It is a cause of severe morbidity and poses a therapeutic challenge for the clinician. Infliximab (Remicade(R); Centocor, Malvern, PA, USA) is a chimeric monoclonal antibody directed against tumour necrosis factor-alpha that has been proven to be effective in the treatment of inflammatory bowel disease (IBD) and rheumatoid arthritis. Currently, very few reports exist documenting its use in the treatment of PG and none in the treatment of PPG. We describe our experience of treating three patients with IBD-associated PPG with infliximab. All patients tolerated the drug without significant side-effects. Two patients with PPG recovered completely following the administration of infliximab, and one patient had a partial response to the drug. We conclude that infliximab appears to be a safe and effective therapeutic alternative in patients with PPG.