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[Postoperative pyoderma gangrenosum developing after cholecystectomy and placement of a stoma].

Pyoderma gangrenosum is a serious necrotizing skin disease frequently associated with diseases of internal organs. In rare instances it may develop after surgical operations or minor injuries causing traumatization of the skin. Our patient developed the disease immediately after cholecystectomy at the site of the scar on the abdomen, and disseminated foci developed also at other sites of the body. In the second patient it developed in the region of the stoma soon after its establishment during development of ulcerative colitis. Skin manifestations healed after systemic combined immunosuppressive treatment.

Adult↗

Pyoderma gangrenosum associated with paroxysmal nocturnal hemoglobulinuria and monoclonal gammopathy.

Pyoderma gangrenosum developed in a man with a five-year history of paroxysmal nocturnal hemoglobinuria and monoclonal gammopathy. He had multiple walnut sized ulcers on his back and extremities, plasma IgM-k type M-protein and low erythrocytic CD55 expression. This is an extremely rare association. However, clonal expansion of plasma cells and chimeric expression of hematopoietic cell glycosylphosphatidylinositol (GPI)-anchored proteins may represent somatic mutations of hematopoietic stem cells in PG as well as PNH. PNH is based on abnormalities in the GPI-anchor formation on various hematopoietic and non-hematopoietic cells. Since the GPI-anchored proteins have pleiotropic functions in complement mediated cell lysis, leukocyte motility, and coagulation systems, the present case may indicate the possible involvement of a GPI-anchored protein abnormality in the pathogenesis of PG.

Biopsy, Needle↗

Treatment of pyoderma gangrenosum with etanercept.

UNLABELLED: We describe a patient with pyoderma gangrenosum (PG) whose lesions responded to etanercept therapy. This disease has been recognized for diverse underlying pathology and associated immune disturbances. Although the role of cytokines in pathogenesis is not fully understood, tumor necrosis factor alpha (TNF-alpha) may facilitate induction and maintenance of the disease. This is supported by the successful use of infliximab, a recombinant anti-TNF-alpha monoclonal antibody, in cases of PG associated with inflammatory bowel disease (IBD). Etanercept is a divalent recombinant fusion protein that binds soluble TNF-alpha. To our knowledge, the utility of etanercept for PG has not been reported. A patient with recalcitrant and widespread PG that was unresponsive to systemic corticosteroids was treated with etanercept. Rapid and complete clearing of the skin lesions was observed, and steroid taper to 5 mg/day was sustained for two months. Treatment was well-tolerated with no adverse reactions reported. CONCLUSIONS: Etanercept therapy offered rapid and complete resolution of all PG lesions. Such response supports the use of etanercept as a steroid-sparing agent in recalcitrant disease and suggests the role of TNF-alpha in pathogenesis of PG.

Adult↗

Parastomal pyoderma gangrenosum: clinical features and management.

BACKGROUND: The importance of pyoderma gangrenosum (PG) as a cause of ulceration around abdominal stomas is not well recognized. OBJECTIVE: Our purpose was to describe the incidence, clinical and histologic features, disease associations, and possible risk factors for parastomal PG. METHODS: A clinic, run by a dermatologist and two stoma nurses, was created. Five hundred patients approached by postal questionnaire were invited to attend if they had skin problems. In addition, local surgical, dermatologic, and nursing services were invited to refer patients with parastomal skin problems. Cases of parastomal PG were identified, investigated, and treated. RESULTS: The annual incidence of parastomal PG in the questionnaire-based cohort of patients was 0.6% (3 patients). An additional 23 patients with the condition were seen. No consistent hematologic, biochemical, immunologic, microbiological or histologic abnormalities were identified. Local skin damage did not appear to be an important trigger for parastomal PG. The condition is recurrent in one third of cases. Topical tacrolimus (0.3% in carmellose sodium paste) has been effective in 4 patients. CONCLUSION: Parastomal PG is far more common than previous reports would suggest, and it may be associated with diseases other than inflammatory bowel disease.

Abdomen↗

Infliximab for the treatment of pyoderma gangrenosum: a randomised, double blind, placebo controlled trial.

BACKGROUND: Pyoderma gangrenosum (PG) is a chronic ulcerating skin condition that often occurs in association with inflammatory bowel disease. There have been a number of reports of PG responding to infliximab, a monoclonal antibody against tumour necrosis factor alpha. AIM: In the first randomised placebo controlled trial of any drug for the treatment of PG, we have studied the role of infliximab in this disorder. SUBJECTS: Patients 18 years of age or older with a clinical diagnosis of PG were invited to take part. METHODS: Patients were randomised to receive an infusion of infliximab at 5 mg/kg or placebo at week 0. Patients were then assessed at week 2 and non-responders were offered open labelled infliximab. The primary end point was clinical improvement at week 2, with secondary end points being remission and improvement at week 6. RESULTS: Thirty patients were entered into the study. After randomisation, 13 patients received infliximab and 17 patients received placebo. At week 2, significantly more patients in the infliximab group had improved (46% (6/13)) compared with the placebo group (6% (1/17); p = 0.025). Overall, 29 patients received infliximab with 69% (20/29) demonstrating a beneficial clinical response. Remission rate at week 6 was 21% (6/29). There was no response in 31% (9/29) of patients. CONCLUSIONS: This study has demonstrated that infliximab at a dose of 5 mg/kg is superior to placebo in the treatment of PG. Open label treatment with infliximab also produced promising results. Infliximab treatment should be considered in patients with PG.

Adult↗

Bullous pyoderma gangrenosum as a manifestation of leukemia cutis.

The authors present the case of a 67-year-old patient in whom bullous pyoderma gangrenosum was the first symptom of acute myeloid leukemia. Histologically leukemic cells were found in the skin infiltrate, on the basis of which this lesion satisfied the criteria of leukemia cutis. It was underlined that in the background of such atypical bullous cases there are often hemoblastoses or their malignant transformation. Finally the connection between bullous pyoderma gangrenosum and atypical vesiculous Sweet syndrome is discussed.

Acute Disease↗

Pathergic pyoderma gangrenosum in a venous ulcer.

A 74-year-old woman was referred to our department in December 1999 for a pyoderma gangrenosum (PG) arising at the edges of chronic leg ulcer. The history was positive for benign monoclonal gammopathy, ischemic hypertensive cardiopathy, polyarthrosis and venous lower leg deficiency. Monoclonal gammopathy of IgA Kappa type was diagnosed 10 years before with a continued benign nature. In 1990 a post traumatic PG of the left leg was diagnosed and a therapy with Cyclosporine A was started with healing of the lesion. In June 1999, 6 months before the hospitalization, a typical venous ulcer of the right leg appeared and was treated with bed-rest, compression bandaging and topical desloughing therapy. In the last month, after a minor surgical debridement of the wound, the lesion developed pustules evolving into a painful, necrotic ulcer with ragged, purple-red, undetermined borders (Fig. 1). A relapse of PG was suspected. Histological examination was consistent with pyoderma gangrenosum and showed massive neutrophilic infiltration, hemorrhage and necrosis of the overlying epidermis. Wound culture was negative. Other laboratory examinations only showed IgA = 8.15 g/L. Investigation of other underlying medical conditions were normal or negative. The venous leg ulcer gradually healed with antiseptic and compression-bandage therapy. After a 4-month course of topical steroid therapy with good results, the PG recurred also involving the proximal area of the leg. Methylprednisolone 50 mg/day was started. Healing began 10 days later and 2 months later the wound healed and epithelialized. Steroid was reduce to 5 mg daily for 4 months. No recurrence was seen when the drug was stopped.

Aged↗

Pyoderma gangrenosum of the perineum.

A case of pyoderma gangrenosum of the perineum is presented. The patient's surgical treatment, hospital course, and 10-year follow-up are described. Unusual problems in grafting, perineal support, and patient emotional response are discussed.

Adult↗

Pyoderma gangrenosum, polycythemia rubra vera, and the development of leukemia.

A patient with long-standing, well-controlled polycythemia rubra vera developed recurrent episodes of bullous pyoderma gangrenosum followed by the transformation of his hematologic disease into a rapidly progressive acute myeloid leukemia. This case, together with previously described patients, indicates that the appearance of bullous pyoderma gangrenosum in a patient with polycythemia rubra vera is often of ominous prognostic significance.

Humans↗

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↗