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Pyoderma gangrenosum of the oral cavity, nose, and larynx.

Pyoderma gangrenosum is a clinical condition often associated with internal disorders and immunologic abnormalities. The diagnosis is deduced solely from clinical examination because no histologic or laboratory changes are pathognomonic. The lesions are quite painful and can be particularly disabling. Laryngeal involvement should elicit concerns for protection of the airway. This case demonstrated involvement of the oral cavity, nose, and larynx--as well as other skin sites of the body--with pyoderma gangrenosum. To our knowledge, this is the first reported case of intranasal and laryngeal pyoderma gangrenosum. Early treatment of any associated internal disorders and aggressive treatment with corticosteroids usually result in adequate control of this problem.

Biopsy↗

Pyoderma gangrenosum treated with hyperbaric oxygen therapy.

The management of pyoderma gangrenosum often requires systemic drug therapy, such as corticosteroids, sulfones, or immunosuppressants, either alone or in combination. Inconsistent response to therapy is a source of frustration to both patient and physician. Several reports in the literature document the successful treatment of pyoderma gangrenosum with hyperbaric oxygen therapy. In our patient, a woman with severe rheumatoid arthritis and diabetes mellitus, hyperbaric oxygen therapy not only promoted healing of pyoderma gangrenosum but permitted reduction of systemic corticosteroids.

Arthritis, Rheumatoid↗

Pyoderma gangrenosum and adrenocortical carcinoma.

Systemic disorders that have been associated with pyoderma gangrenosum include inflammatory bowel disease, rheumatoid arthritis, paraproteinemias, and hematologic malignancies. We report the case of a 55-year-old woman with pyoderma gangrenosum, IgA monoclonal gammopathy, and a cortisol-secreting adrenocortical carcinoma. Review of the literature revealed one previous case of pyoderma gangrenosum associated with a solid tumor; at autopsy, a carcinoid tumor and an adrenocortical adenoma were found. Our patient's rapid improvement after the carcinoma was resected and her subsequent disease-free course suggests that the two conditions were related. This case suggests that evaluation for underlying malignancy should be considered in patients with pyoderma gangrenosum.

Adrenal Cortex Neoplasms↗

Pyoderma gangrenosum in an HIV-infected patient.

Pyoderma gangrenosum has previously been reported in four patients with HIV infection. We describe here the case of a fifth HIV-infected patient with pyoderma gangrenosum and review the characteristics of the four previously reported cases.

Adult↗

Pyoderma gangrenosum: recognition and management.

Pyoderma gangrenosum, a progressive necrotizing and ulcerative disease of the skin, presents serious problems in diagnosis and management to physicians not familar with the entity. The clinical basis of the diagnosis is outlined, and the possible etiological mechanisms are reviewed. Treatment is primarily nonsurgical, relying on oral administration of corticosteroids and conservative local care of the ulcers.

Diagnosis, Differential↗

Pyoderma gangrenosum and related disorders.

Pyoderma gangrenosum (PG) is an uncommon ulcerative cutaneous condition with distinctive clinical characteristics and a frequent association with systemic diseases. Most frequently among the associated disease list are: inflammatory bowel disease, arthritis, and a variety of hematologic disorders. There is no specific treatment of PG; however, the effective treatment of an accompanying systemic disease, local therapy, and/or the use of topical or systemic agents known to be useful in neutrophil disorders, have been beneficial in most patients.

Humans↗

Serum C-reactive protein measurement in pyoderma gangrenosum.

A case of pyoderma gangrenosum is reported in a patient with a past history of ulcerative colitis. Serial C-reactive protein (CRP) concentrations were measured and were found to be markedly elevated at presentation. Levels were also raised during two subsequent relapses. CRP levels proved to be a useful objective indicator of disease activity and response to therapy, and reflected changes in disease activity before the erythrocyte sedimentation rate.

C-Reactive Protein↗

Pyoderma gangrenosum involving the foot. A case report.

Pyoderma gangrenosum is a rare and destructive inflammatory skin disease. The authors present a report of a patient with a classic case of pyoderma gangrenosum involving the foot. The diagnosis was made on the basis of clinical presentation and progression of the disease after differential diagnoses of common conditions were excluded. A brief overview of the disease process, its treatment, and its correlation with ulcerative colitis is provided.

Adrenal Cortex Hormones↗

Pyoderma gangrenosum at the parastomal site in patients with Crohn's disease.

Pyoderma gangrenosum is a rare idiopathic skin condition often associated with other diseases, including Crohn's disease. We believe this is the first report of cases in which pyoderma gangrenosum was identified at the parastomal site in patients with Crohn's disease. Four episodes of the condition occurred in three patients. In these patients, pyoderma gangrenosum first appeared after two to three surgical procedures and between ten months and five years after the initial surgery. The disease course varied from resolution within two months with corticosteroid therapy to resolution only after revision or relocation of the stoma with resection of recurrent disease segments. Parastomal pyoderma gangrenosum at the ileostomy site in patients with Crohn's disease may be more common than previously thought. Optimal treatment has not yet been established, and treatment needs to be tailored for each patient.

Adult↗

Clofazimine in the treatment of pyoderma gangrenosum.

Ten patients with pyoderma gangrenosum, seven female and three male, 25 to 94 years old, mainly with multiple lesions, have been treated with clofazimine, 100 mg three times daily. Associated disease was registered in three patients: diabetes mellitus, a previous adenocarcinoma of the colon treated by hemicolectomy, and pustulosis palmoplantaris. In a further patient, M-component was found in the serum. In seven cases the lesions were completely healed by two to five months of therapy, and in three cases the ulcers healed partially. Side effects were redness of the skin in seven cases and dryness of the skin in two patients. No hematological side effects occurred. The working mechanism is still obscure.

Adult↗

Immunologic and biochemical studies on a patient with pyoderma gangrenosum.

A patient with pyoderma gangrenosum without associated disease was studied. Routine investigations showed several abnormalities. High ESR, high alkaline phosphatase and glutamyl transferase (gamma-GT) levels, low iron and iron binding capacity, altered protein spectrum, presence of Staphylococcus aureus and group G hemolytic streptococci in ulcer culture, higher than normal antistreptolysin titers in the serum, and perivascular infiltration in the skin. Biochemical investigations aimed at finding any excessive hydrolytic activity did not reveal the presence of neutral proteases in circulation leaked out from PMN-leukocytes or elsewhere. Lysozyme levels were higher than normal, amylase and lipase levels were normal and 5' nucleotidase levels were below normal range. TCA-soluble polypeptides were present in the serum at levels two times higher than those in normal individuals. Immunochemical investigations showed the absence of immune complexes in the serum but presence of high amounts of C-reactive protein. Total complement activity was higher than normal and so was C3c level. Clq, C4, and C3d levels were within normal range. Biologic studies showed the presence of a factor in patient serum that made guinea pig skin hard, painful, erythematous, and eventually hairless, but not necrotic. A similar factor was either absent in normal serum or present in very low concentration. After salazopyrine treatment, all the above mentioned abnormalities corrected except that 5' nucleotidase activity remained slightly lower than normal, alkaline phosphatase levels remained slightly higher than normal, and C-reactive protein levels remained very high, though lower than those during intense disease activity.

Drug Combinations↗

Treatment of pyoderma gangrenosum.

Critical to the proper management of pyoderma gangrenosum are correct diagnosis, identification and treatment of any underlying disorder, and the proper choice of topical and systemic therapy. Many agents are available for the treatment of pyoderma gangrenosum. We review the current therapeutic options, their efficacy and side effects, and we offer some guidelines for their proper selection.

Administration, Cutaneous↗

Pyoderma gangrenosum and extensive caval thrombosis associated with the antiphospholipid syndrome--a case report.

Pyoderma gangrenosum is a rare cutaneous disorder frequently associated with systemic diseases. The authors report the case of a 28-year-old man with pyoderma gangrenosum and inferior cava vein syndrome due to thrombosis. The search for pyoderma gangrenosum-associated conditions (gastrointestinal, rheumatic, neoplasic, and infectious diseases) was negative. A prolonged partial thromboplastin time and a positive test for circulating anticardiolipin antibodies strongly suggested the diagnosis of the antiphospholipid syndrome. There are reports of at least three other cases of pyoderma gangrenosum associated with the antiphospholipid syndrome. This clinical case adds further evidence to the possible link between these disorders and warrants a search for anticardiolipin antibodies in patients with pyoderma gangrenosum.

Adult↗

Gigantic pyoderma gangrenosum.

A 71-year-old Japanese female with gigantic pyoderma gangrenosum is reported. The pyoderma lesions had been treated as an infectious condition for seventeen months and had extended to enormously large areas. The nature of the chronic type of pyoderma gangrenosum may need to be stressed, even for dermatologists.

Aged↗

Split skin grafts in the treatment of pyoderma gangrenosum. A report of four cases.

BACKGROUND: Pyoderma gangrenosum (PG) is an uncommon necrotising, non-infective ulceration of the skin. The management of PG is aimed at limiting tissue destruction, promoting the healing of the wound, and providing an acceptable cosmetic result. However, skin grafting is normally avoided because of the potential risk of pathergy-the localization of skin damaged by trauma. REPORT: We describe the use of split skin grafts in the management of ulcerative pyoderma gangrenosum in 4 patients. RESULTS: Our cases demonstrate that split skin grafts are a useful treatment modality in patients with ulcerative PG, producing a good cosmetic result. One case illustrates the importance of ensuring the disease is quiescent prior to grafting, to avoid pathergy. The other cases emphasise the need for prolonged immunosuppressive therapy to minimise the chance of reactivation of the disease process. CONCLUSION: Our preliminary experience of 4 cases of ulcerative PG indicates that split skin grafts have a role to play in its management. The ultimate cosmetic result is considered to be superior to allowing the wound to heal by secondary intention. To limit the risk of pathergy developing, our experience suggests a role for prolonged courses of immunosuppressive therapy. The most effective dose and duration of immunosuppressive therapy in patients with PG treated with split skin grafts remains to be determined. A controlled study would be of benefit to compare it with other current treatment options.

Abdomen↗

Pyoderma gangrenosum triggered by alpha2b-interferon in a patient with chronic granulocytic leukemia.

Pyoderma gangrenosum is a neutrophilic dermatosis that is frequently associated with malignancies such as myeloproliferative disorders. The development of this dermatologic disorder is thought to be mediated by immunological mechanisms. A case of pyoderma gangrenosum associated with the administration of alpha2b-interferon (alpha2b-IFN) in a patient with chronic granulocytic leukemia is described. Discontinuation of alpha2b-IFN and the administration of cyclosporin A and prednisone resulted in cure of the pyoderma gangrenosum. Serum levels of tumor necrosis factor, interleukin-6 and soluble interleukin-2 receptor increased when the cutaneous lesions appeared and returned to normal levels when the lesion healed. We believe that this is the first reported case of pyoderma gangrenosum associated with alpha2b-IFN therapy.

Antineoplastic Agents↗

Peristomal pyoderma gangrenosum.

A patient with Crohn's disease and peristomal pyoderma gangrenosum is described. This patient is unique because she had a rapid response to intralesionally injected steroids. This treatment is ideal for peristomal pyoderma gangrenosum because it is administered intermittently when the ostomy appliance is changed and it does not interfere with adhesion of the device. All 11 cases of peristomal pyoderma gangrenosum described in the literature are reviewed.

Abdominal Muscles↗

Pyoderma gangrenosum in pediatric acquired immunodeficiency syndrome.

We describe two children with human immunodeficiency virus infection in whom pyoderma gangrenosum developed. Although pyoderma gangrenosum most commonly occurs in children with inflammatory bowel disease, it has also been described in patients with a variety of immunodeficiencies. In such patients a vigorous search to exclude a treatable infection should be made before the lesions are treated as pyoderma gangrenosum.

Acquired Immunodeficiency Syndrome↗