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[Pseudo-septicemic pyoderma gangrenosum and breast cancer. Apropos of a case caused by an intramuscular injection].

We report a case of pyoderma gangrenosum (PG) mimicking a severe infectious skin disease in a woman with metastatic breast cancer. PG started at the site of an intramuscular injection administered a few days previously, and it subsequently extended. The skin disease was cured by high-dose corticosteroid therapy and clofazimine, but it marked a turn for the worst in the course of the breast cancer which became rapidly fatal.

Breast Neoplasms↗

Pyoderma gangrenosum presenting as an acute suppurative hand infection--a case report.

Pyoderma gangrenosum, an uncommon disorder characterized by necrosis and noninfective skin ulcerations, was found in a patient who presented with an apparent hand infection. Cultures of the purulent material showed no growth. The treatment of choice was large doses of steroids and control of his ulcerative colitis. No similar case could be found in the literature.

Acute Disease↗

Management of pyoderma gangrenosum.

The management of pyoderma gangrenosum (PG) requires a structured approach to establishing diagnosis of the disease and assessment of the patient. Clinical management of active PG lesions should be carried out in coordination with other specialists (such as nurses and pain managers) and often necessitates a flexible, innovate attitude to therapy, because the needs of individual patients may vary widely. Although there is no single successful treatment for this disease, certain types of PG lesions are recognized to respond more readily to accepted therapies than others. We outline guidelines to the management of the patient with PG and discuss alternative therapies.

Humans↗

A large outbreak of streptococcal pyoderma in a military training establishment.

An outbreak of streptococcal pyoderma in a military institution into which fresh susceptible recruits were regularly introduced involved more than 1300 persons over an eighteen-month period. Two M types were responsible for the great majority of the cases and an attack by one conferred immunity to that strain but not to the other. Lesions varied from trivial to disabling. Epidemiological studies indicated that contact -- direct or indirect -- through such things as gymnasium equipment and room dust was the means of transmission. The outbreak was eventually controlled by vigorous case finding, thorough treatment of cases and the strict application of hygienic principles to prevent spread. There were no late complications and throat streptococci were not involved. It is possible to control such an outbreak without isolation or the making of any significant concessions in the training programme even when large numbers of persons are living and working at close quarters.

Follow-Up Studies↗

Treatment of pyoderma gangrenosum with infliximab in Crohn's disease.

Pyoderma gangrenosum (PG) is an ulcerating noninfectious disease of the skin seen in 1 to 5% of patients with inflammatory bowel disease. The pathogenesis of PG has yet to be determined but may be related to abnormal T cell responses and the production of TNF-alpha, a powerful proinflammatory cytokine. Infliximab, a chimeric monoclonal antibody to TNF-alpha, has been approved for the treatment of Crohn's disease. We present four patients with PG treated with Infliximab for fistulizing Crohn's in whom complete healing of PG was achieved. Four patients with active fistulizing Crohn's disease and PG were treated. All patients were females ranging in age from 48 to 60 years, with a mean age of 54 years. Three of four patients had PG lesions located on the lower extremities; one patient had peristomal disease. All patients had at least colonic involvement of their Crohn's. The patients received either a single infusion or a series of three 5 mg/kg Infliximab infusions. All four patients demonstrated rapid healing of PG within 4 weeks of the first infusion of Infliximab. PG healing followed improvement in bowel disease. Complete resolution without recurrence was noted in all patients. Rapid resolution of PG was noted in four female patients with fistulizing Crohn's disease treated with Infliximab. Healing was complete, without recurrence. The anti-TNF-alpha properties of Infliximab suggest that healing may be mediated by the drug's effect on cytokine pathways, perhaps by blunted T cell activation early in the inflammatory cascade. We suggest an independent effect of Infliximab on PG.

Adult↗

Giant cells in pyoderma gangrenosum.

It has been claimed that pyoderma gangrenosum (PG) lesions may contain granulomatous foci when associated with Crohn's disease. To test this assertion, we obtained clinical histories and archived cutaneous biopsies from 34 PG patients. Thirteen of these patients had inflammatory bowel disease (IBD). Immunostaining with PGM1, a macrophage marker, revealed well-formed giant cells with three or more nuclei in biopsies from 6 of 13 patients with IBD. Five of the 6 biopsies came from patients with Crohn's disease and one from a patient with ulcerative colitis. Two were peristomal. In the 21 patients who had PG without IBD, no giant cells were seen. Thus, PGM1+ histiocytic giant cells within a PG lesion may be indicative of associated IBD (p = 0.006), particularly Crohn's disease.

Crohn Disease↗

Pyoderma gangrenosum: a review.

Since its first description in 1930, the pathogenesis of pyoderma gangrenosum (PG) has remained obscure even as an ever-widening array of systemic diseases has been described in association with it. The histopathologic distinction of PG from other ulcerative processes with dermal neutrophilia is challenging and at times impossible. In consequence, when confronted with a biopsy from such a lesion, the pathologist has an obligation to obtain a full and detailed clinical history. In short, as a diagnosis of PG does not hinge exclusively upon the biopsy findings in isolation from other studies, a solid knowledge of the clinical features, the systemic disease associations and the differential diagnosis will help the pathologist to avoid diagnostic pitfalls or the generation of a report which is non-contributory to patient care. In this review, we describe in detail the different clinicopathologic forms of PG, summarize the diseases associated with this process in the literature and in our experience, and briefly review the treatment options.

Adult↗

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↗

Association of pyoderma gangrenosum and sterile osteomyelitis in a patient having myelodysplastic syndrome with der(1;7)(q10;q10).

Neutrophilic dermatoses such as Sweet's disease and pyoderma gangrenosum (PG) are occasionally associated with myelodysplastic syndrome (MDS). We present here a 67-yr-old male having PG and sterile osteomyelitis in association with underlying MDS (refractory anemia) and Crohn's disease. To establish the diagnosis of MDS, sternal bone marrow puncture was performed, which showed chromosomal abnormality containing der(1;7)(q10;q10). After the puncture, he suffered from gradually progressive skin ulceration, flare, and bone pain. Magnetic resonance imaging (MRI) of the sternum showed severe inflammation in the sternum and the overlying subcutaneous tissue. All of the cultures obtained from the wound were negative for both bacteria and fungus. Biopsy was performed from the antero-sternal skin lesion, which showed epidermal ulceration with prominent infiltration of neutrophils. He was thus diagnosed as having PG and sterile osteomyelitis, and was treated with prednisolone, which completely resolved the symptoms. We consider that the bone marrow aspiration in the present patient provoked PG and sterile osteomyelitis. As was previously reported by others, certain chromosomal abnormalities in MDS may be related with the development of neutrophilic dermatoses.

Aged↗

Use of computerized image analysis to quantify staphylococcal adhesion to canine corneocytes: does breed and body site have any relevance to the pathogenesis of pyoderma?

An optimized system of computerized image analysis was used to investigate variations in the adherence of Staphylococcus intermedius to canine corneocytes from four different breed groups and six different anatomical sites. S. intermedius showed significantly greater adherence to the head and neck compared with the dorsum, but adherence to the limb, axilla and groin did not differ from other sites. Furthermore, there was significantly greater adherence of S. intermedius to corneocytes from the dorsum, forelimb, axilla and groin of Boxers and Bull Terriers than Spaniels and Hounds. S. intermedius, and also Pseudomonas aeruginosa, exhibited abundant adherence, which was significantly greater than Staphylococcus aureus, Streptococcus canis, Klebsiella pneumoniae and Escherichia coli. In addition, S. intermedius adherence demonstrated a sigmoid dose-response curve with increasing bacterial concentration. These results suggest that S. intermedius adheres to canine corneocytes by a specific receptor-ligand interaction and adheres to the skin of some breeds more avidly than others. However, variations in adherence between body regions would not account for the predilection sites of canine bacterial pyoderma.

Animals↗

Pyoderma gangrenosum of the penis.

Two cases of pyoderma gangrenosum involving the penis are presented. Treatment was difficult in both cases despite the use of high doses of prednisolone. In one case, the addition of thalidomide proved successful, whereas in the other, minocycline effected a cure.

Adult↗

Pyoderma gangrenosum in a child with congenital partial deficiency of leucocyte adherence glycoproteins.

Congenital deficiency of beta 2 integrin leucocyte adhesion molecules is a rare immunodeficiency and is often fatal. Neutrophils are unable to bind to ligands on the endothelium, and so cannot leave the circulation during inflammation or infection. When leucocyte adhesion deficiency (LAD) is caused by abnormally low expression of beta 2 integrins, it is termed LAD type 1. We describe a 5-year-old girl with a history of recurrent bacterial infections since early childhood who developed necrotic skin ulcers resembling pyoderma gangrenosum and a persistent circulating neutrophilia. Histologically, the lesions showed deep ulceration with a diffuse lymphohistiocytic infiltrate, but with a relative sparsity of neutrophils. Subsequent investigation revealed a complete absence of CD11a/CD18 beta 2 integrins on the surface of the patient's neutrophils, confirming the diagnosis of LAD type 1. The ulcers responded to treatment with oral prednisolone and colchicine.

CD11 Antigens↗

Mycosis fungoides bullosa simulating pyoderma gangrenosum.

A patient with mycosis fungoides (MF) bullosa had a rapidly growing, painful necrotic mass on the left ankle which extended by peripheral bulla formation, clinically resembling pyoderma gangrenosum. Histopathology confirmed MF bullosa with both intraepidermal and subepidermal bulla formation.

Antineoplastic Agents, Phytogenic↗

Severe recalcitrant pyoderma gangrenosum responding to a combination of mycophenolate mofetil with cyclosporin and complicated by a mononeuritis.

We describe a 17-year-old boy with severe recalcitrant pyoderma gangrenosum. Healing was achieved with a combination of mycophenolate mofetil and cyclosporin, negative pressure dressings and split-skin grafts. His recovery was complicated by a sciatic nerve palsy, which we believe was caused by direct involvement of the nerve at the level of the sciatic notch.

Adolescent↗

Pyoderma vegetans and ulcerative colitis.

Pyoderma vegetans (PV) is a chronic, vegetating pustular disorder characterized clinically by erythematous vesiculopustular vegetating cutaneous plaques. Marked epidermal hyperplasia, intraepidermal and subepidermal neutrophilic microabscesses and a dermal inflammatory infiltrate are the prominent histopathological findings. We describe a patient with PV associated with ulcerative colitis and mammary Paget's disease. Pustular eruptions associated with ulcerative colitis are reviewed.

Breast Neoplasms↗