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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

Selective immunoglobulin M (IgM) deficiency in two immunodeficient adults with recurrent staphylococcal pyoderma.

Two adult men with recurrent pyoderma due to Staphylococcus aureus and a selective deficiency of immunoglobulin M (IgM) antibody synthesis are described. An analysis of each patient's polymorphonuclear leukocyte chemotaxis, phagocytosis and killing of Staph. aureus, serum opsonizaiton of Staph. aureus, and serum and lymphocyte-mediated responses to antigenic stimulation was performed. Family studies revealed a possible autosomal dominant inheritance pattern with heterogenetic expression of various dysgammaglobulinemic states in each patient's first degree relatives. In vivo studies of delayed hypersensitivity and in vitro studies of polymorphonuclear leukocyte and lymphocyte function were normal. A defect in IgM, but not in IgG (immunoglobulin G), antibody synthesis to a number of antigens, and a mild decrease in serum opsonic activity to Staph. aureus correctable by heat inactivated normal human serum were found in each patient. In these patients, the recurrent staphulococcal pyoderma prompted an investigation of host defense mechanisms and revealed low to absent IgM levels and a defect in IgM antibody synthesis.

Adult

Pyoderma gangrenosum associated with systemic lupus erythematosus: response to pulse steroid therapy.

Pyoderma gangrenosum in a 35-year-old woman with long-standing systemic lupus erythematosus was treated with pulse steroid therapy. Significant improvement in the ulceration and control of the lupus erythematosus occurred. The unusual association of these disorders is reviewed, and the efficacy of pulse therapy in the treatment of refractory cases of pyoderma gangrenosum is 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-like ulcer in a patient with large granular lymphocytic leukemia.

Large granular lymphocytic leukemia refers to a clonal expansion of lymphocytes that have abundant cytoplasm and azurophilic granules. The disease is characterized clinically by chronic neutropenia and it may be associated with recurrent pyogenic infections. Except for these infections, cutaneous manifestations of this disease have not been well characterized. We describe a patient with large granular lymphocytic leukemia, which was confirmed by molecular genetics studies, who had a pyoderma gangrenosum-like ulcer on his leg. Results of an evaluation of the histologic characteristics and the leukocytic immunophenotype of a skin biopsy specimen from the ulcer demonstrated large granular lymphocytes within the blood vessels. Cutaneous ulceration may be a manifestation of large granular lymphocytic leukemia, and this disease should be considered when diagnosing patients with otherwise unexplained pyoderma gangrenosum-like ulcers of the skin.

Complement C3

Pyoderma gangrenosum presenting as Fournier's gangrene.

We report a case of pyoderma gangrenosum presenting as Fournier's gangrene. Although both processes have a similar presentation effective management is markedly different. Whereas broad-spectrum antibiotics and aggressive surgical débridement are necessary to control Fournier's gangrene, immediate institution of corticosteroids and local wound care are indicated for pyoderma gangrenosum.

Adult

Scrum kidney: epidemic pyoderma caused by a nephritogenic Streptococcus pyogenes in a rugby team.

In December, 1984, an outbreak of pyoderma affected five scrum players in the St Thomas' Hospital rugby team. The causative organism, Streptococcus pyogenes, was acquired during a match against a team experiencing an outbreak of impetigo, and was transmitted to two front row players of another team a week later, and to two girlfriends of affected St Thomas' players a month later. The strain was M-type 49, tetracycline-resistant, and virulent. It caused salpingitis in a girlfriend and acute glomerulonephritis in one rugby player. No case of subclinical glomerulonephritis was detected in eight patients with pyoderma. Screening of the St Thomas' Hospital team revealed four further cases of non-streptococcal skin infection, with evidence for contemporaneous spread of Staphylococcus aureus. Teams should not field players with sepsis, and it may be advisable to apply a skin antiseptic to traumatised skin after the match.

Acute Disease

Pyoderma gangrenosum and progressive cutaneous ulceration.

Pyoderma gangrenosum is a cutaneous disorder characterized by slowly progressive ulceration which is refractory to local wound care and antibiotic therapy. The diagnosis is made after the exclusion of other causes of cutaneous ulceration. The cause of pyoderma is thought to be a deficiency in host immune reactivity, and a systemic illness is present in 80% of patients. High-dose corticosteroids and appropriate treatment of the underlying disease represent the mainstays of therapy, although hyperbaric oxygen has recently shown promise in the healing of skin grafts over these lesions.

Adult

Intralesional steroid therapy of pyoderma gangrenosum.

Treatment of pyoderma gangrenosum complicating ulcerative colitis has in the past been frustrating and usually unsuccessful, frequently resulting in colectomy. Intralesional steroid therapy has been used off and on in a few patients, but the treatment is not widely known to gastroenterologists. We report two patients successfully treated for early pyoderma gangrenosum by intralesional injection of triamcinolone acetonide (Kenalog, 40 mg injection) together with conventional doses of systemic steroids. A single series of injections was sufficient to bring about healing of the lesions in both patients. The treatment permitted early discharge of patients from the hospital, and the skin lesions were completely healed within 2 months. The lesions have not recurred in follow-up examinations of 11/2 and 21/2 years, respectively. We hope that other physicians will find this approach equally effective.

Adolescent

Rapid antigen detection in the diagnosis of group A streptococcal pyoderma: influence of a "learning curve effect" on sensitivity and specificity.

To evaluate the efficacy of rapid antigen detection for diagnosis of Group A streptococcal pyoderma, lesions on 129 children were swabbed for culture and for rapid antigen detection testing. Duplicate cultures and duplicate rapid antigen tests were performed in two laboratories. The sensitivity and the specificity of the test were high, supporting the validity of this technique in diagnosing streptococcal pyoderma. Of importance, however, was the documentation of a learning curve effect on the sensitivity and specificity. There was a significant difference between the early samples and the later samples in one laboratory which had less previous experience with the rapid antigen detection test. By the time the later samples were collected, the proficiency of the first laboratory was equal to that of the second laboratory for both sensitivity and specificity. Although a learning curve effect on sensitivity and specificity of rapid antigen detection tests for Group A streptococci has been suspected, this is the first documentation of such an effect. This finding has important clinical implications for using rapid antigen detection tests for Group A streptococcal infections.

Antigens, Bacterial

Pyoderma gangraenosum (dermatitis ulcerosa) and monoclonal (IgA) globulin healed after melphalan treatment. Case report and review of the literature.

Since 1968 we have been treating a patient, who has had a combination of pyoderma gangraenosum (dermatitis ulcerosa) and signs that may indicate early multiple myeloma. She also had carcinoma of the colon, which was successfully operated. The pyoderma healed later after intensive and successful cytostatic treatment of the "myeloma". The ulcers remain practically healed and the protein pattern is normal in May 1977. Such cases are rare and a search in the literature has not been very rewarding. In our own series of more than 200 cases with myeloma this combination is unique. The lieterature is discussed in detail with data on the follow-up on some of the patients.

Aged

Malignant pyoderma.

A patient is described who developed multiple areas of inflammatory pyoderma on the face, leading to extensive ulceration. Repeated investigations failed to demonstrate any specific bacteria or fungus responsible and trials of treatment with various antibiotics proved unsuccessful. Histology revealed a granulomatous abscess-like lesion, and the condition resembled what has been described as malignant pyoderma. The patient was successfully treated with oral dapsone and intralesional steroids.

Dapsone

Clinical relevance of the antibacterial activity of terbinafine: a contralateral comparison between 1% terbinafine cream and 0.1% gentamicin sulphate cream in pyoderma.

The antibacterial efficacy of 1% terbinafine cream and 0.1% gentamicin sulphate cream was evaluated in 33 patients with superficial staphylococcal pyoderma in a double-blind contralateral comparison. After 12 days of active treatment, Staphylococcus aureus could be grown from only one out of 33 patients using terbinafine vs no patient using gentamicin. At the end of the study, a marked improvement in clinical symptoms was observed with no significant difference between the two therapies. Despite reviewing the patients three times during the course of the study, no adverse events were reported. The results of this study demonstrate that terbinafine has clinically relevant antibacterial properties which may be useful not only in pyoderma, but also in mixed fungal/bacterial infections, such as athlete's foot.

Adolescent

Pyoderma gangrenosum in an allogeneic bone marrow transplant recipient.

We present the case of a 15-year-old boy who developed facial pyoderma gangrenosum following an allogeneic bone marrow transplantation for the treatment of a 'blast' crisis developing in the course of chronic myelogenous leukaemia. The lesion appeared 7 months before any evidence of relapse. The discussion is focused on both the presentation of pyoderma gangrenosum associated with myelo-proliferative disorders and its pathogenesis via the underlying immunosuppression.

Adolescent

Treatment of pyoderma gangrenosum with cyclosporin A.

Two patients with recalcitrant pyoderma gangrenosum were treated with oral cyclosporin A (5 mg/kg body-weight/day). Healing of the lesions was achieved in Patient 1 within 1 month of starting treatment, but new areas of ulceration appeared when the dose was reduced to 3 mg/kg body-weight/day. The ulcers showed marked improvement by 3 weeks after the start of treatment in Patient 2 and remained inactive at a maintenance dosage of 100 mg/day, but there was no change in the associated seronegative arthritis. A steroid-sparing effect of CyA was evident in both patients. It is suggested that a lower dose of cyclosporin A than doses used previously in the treatment of pyoderma gangrenosum may be equally effective.

Adult

Wegener's granulomatosis presenting as pyoderma gangrenosum.

We report three cases of Wegener's granulomatosis presenting with cutaneous ulceration resembling pyoderma gangrenosum. Wegener's granulomatosis classically affects the upper and lower respiratory tracts and the kidneys. Skin involvement occurs in up to 50% of patients. Increased awareness that cutaneous involvement can take the form of pyoderma gangrenosum and that it can be a presenting sign may lead to more rapid diagnosis of Wegener's granulomatosis.

Adult