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Post-surgical pyoderma gangrenosum of the vaginal vault associated with ulcerative colitis and Behçet's disease; a case report.

A case is described in which a patient with ulcerative colitis developed chronic vaginal ulceration around the incision lines after cone biopsy. The ulcers persisted at the vaginal vault after abdominal hysterectomy and removal of a cuff of vagina. Non-specific histological features compatible with pyoderma gangrenosum were found on biopsy. The auto-immune nature of this chronic ulcer is further supported by its rapid response to steroid therapy and the subsequent development of Behçet's syndrome.

Adult↗

Immunologic aspects of German shepherd dog pyoderma (GSP).

In 21 dogs with clinical features of German Shepherd dog Pyoderma (GSP) parameters of the specific and aspecific immune system have been examined. Chemotaxis and killing capacities of neutrophilic leucocytes were undisturbed, whereas in skin biopsies no specific immunoglobulin or complement deposits were found with immunofluorescence. With double immunodiffusion, antibodies against Gram-positive bacteria were found. In a laser nephelometric assay significantly elevated levels of IgG, IgGab, IgGd, IgM and bacterial components, associated and non-associated with circulating immune complexes, were detected. However, no relation was found with the disease state. It is concluded that dogs with GSP are immunologically normal reactors. A bacterial hypersensitivity reaction is hypothesized as a possible initiating factor in the pathogenesis of GSP.

Animals↗

Lymphocyte subset abnormalities in German shepherd dog pyoderma (GSP).

Peripheral blood lymphocyte subpopulations were studied in 12 German shepherd dogs suffering from deep pyoderma (GSP). Twelve other healthy but matched dogs were used as controls. GSP was found to be associated with an imbalance in the CD4 and CD8 subsets (respectively 37.3 +/- 8.7% and 28.6 +/- 6.6%, as compared to 47.5 +/- 8.8% and 19.3 +/- 4.0% in the controls). The activation markers were not affected by GSP. Moreover, analysis of the B-cell populations showed a striking decrease in the level of CD21 cells (5.5 +/- 3.3% of CD21+ lymphocytes, compared to 12.2 +/- 6.0 in the controls). This study suggests that the immunological imbalance observed in GSP may be associated with defective helper cells, and provides further evidence that dogs suffering from GSP are not immunologically normal reactors.

Animals↗

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↗

Cutaneous mucormycosis resembling superficial granulomatous pyoderma in an immunocompetent host.

Primary cutaneous mucormycosis is uncommon and occurs mainly in immunosuppressed or diabetic patients or after localized trauma. We report a case of cutaneous mucormycosis in an immunocompetent man in whom no definite precipitating factors could be identified; initially, superficial granulomatous pyoderma was suspected clinically. The lesion was successfully treated with debridement, local wound care, parenteral administration of amphotericin B, and ultimately by surgical excision and split-thickness skin grafting.

Dermatomycoses↗

Epidemiology and control of staphylococcal pyoderma among newborn infants: evaluation of a method for routine cord care with 4 per cent chlorhexidine-detergent solution.

The incidence and types of staphylococcal purulent lesions were prospectively studied among 3602 newborn infants. During an epidemic of post-discharge pyoderma, 87 per cent of all Staphylococcus aureus isolates from lesions could be traced to the nursery. The risk of post-discharge disease was related to the degree of staphylococcal skin colonization in the nursery. A method for routine cord care in the nursery with 4 per cent chlorhexidine-detergent solution was evaluated and proved to be effective in reducing staphylococcal colonization in the nursery as well as purulent skin disease at home. The clinical effectiveness of the regimen was followed up for 19 months and a persistent low infection rate was obtained among infants in the post-discharge period.

Chlorhexidine↗

Sulfapyridine and sulfones decrease glycosaminoglycans viscosity in dermatitis herpetiformis, ulcerative colitis, and pyoderma gangrenosum.

Shortly after the introduction of sulfa drugs, sulfapyridine was found to have unique therapeutic properties, unrelated to antibacterial activity. Later, sulfones were found to share the same properties. The disorders initially improved were dermatitis herpetiformis, pyoderma gangrenosum, subcorneal pustular dermatosis, acrodermatitis continua, impetigo herpetiformis and ulcerative colitis. They were also sometimes helpful in many other disorders. They are effective in select disorders characterized by edema followed by granulocytic inflammation or edema followed by vesicle or bullae formation. The sulfones work in low doses in leprosy and their mode of action is not fully understood. Several pieces of experimental information are available. It is proposed that these drugs are entering or influencing the protein moiety of glycosaminoglycans and decreasing tissue viscosity. This decreased tissue viscosity prevents edema and dilution of tissue fluid and decreases acute inflammation and vesicle and bullae formation.

Clofazimine↗

Pyoderma eczema and folliculitis with defective leucocyte and lymphocyte function: a new familial immunodeficiency disease responsive to a histamine-1 antagonist.

A new familial immunodeficiency disease characterised by recurrent and persistent pyoderma, folliculitis, and atopic dermatitis is described in a father and son. It is accompanied by abnormalities of lymphocyte function (including defective proliferative responses to phytomitogens, and subnormal response in immunoglobulin production after stimulation of the lymphocytes by pokeweed mitogen) and defective leucocyte chemiluminescence responses, which were associated with defective ability for intracellular killing of microbial organisms. The abnormalities of lymphocyte and leucocyte function, as well as the clinical manifestations, responded dramatically to treatment with the histamine-1 antagonist, chlorpheniramine, suggesting that the underlying defect in this disease may relate to defective histamine metabolism or abnormal expression of histamine receptors on lymphocytes and leucocytes.

Blepharitis↗

Pyoderma gangrenosum and Chlamydia pneumoniae infection in a diabetic man: pathogenic role or coincidence?

Chlamydia Pneumoniae is not a known cause of skin infections, but unusual pathogens cause chronic infections in diabetic patients. Multiple idiopathic pyoderma gangrenosum-like (PG-like) lesions were refractory to multiple therapeutic agents in a diabetic patient who had C pneumoniae identified by serologic tests and polymerase chain reaction. Based on complete resolution by prolonged anti-chlamydial antibiotic therapy and concomitant decrease in serologic and titers determined by polymerase chain reactions, the PG-like lesions were presumed to be due to C pneumoniae.

Aged↗

Pyoderma gangrenosum treated with clofazimine. Report of three cases.

Three cases of pyoderma gangrenosum (PG) responsive to clofazimine are reported. The ulcers were recalcitrant to other forms of therapy, and the patients were incapacitated. Healing began within 2 or 3 weeks. To our knowledge, these are the first reported cases of PG responsive to clofazimine in the United States.

Chronic Disease↗

Pyoderma gangrenosum: successful treatment with intralesional steroids.

A case of pyoderma gangrenosum (PG) responsive to intralesional injection of corticosteroid is reported. Improvement was detected by 48 hours, and complete healing had occurred by 6 weeks. The association with various underlying systemic diseases, pathogenesis, and modalities of therapy are discussed.

Humans↗

Neutrophilic dermatosis of myeloproliferative disorders. Atypical forms of pyoderma gangrenosum and Sweet's syndrome associated with myeloproliferative disorders.

Atypical forms of pyoderma gangrenosum (PG) and Sweet's syndrome (SS) (acute febrile neutrophilic dermatosis) have been separately reported in association with various forms of leukemia over the past decade. A case in which both atypical bullous PG and atypical SS occurred in a patient with myeloid metaplasia is presented, and the literature is reviewed concerning the association of these dermatoses with neoplasias of myeloid origin. The current case and review support the thesis that these dermatoses, when associated with myeloproliferative disorders, represent points on a continuum of noninfectious, nonmetastatic, inflammatory neutrophilic dermatoses that may occur in patients with derangements in myeloid cell proliferation.

Adult↗

Hydralazine-induced systemic lupus erythematosus presenting as pyoderma gangrenosum-like ulcers.

A 48-year-old white woman who for 3 years had been taking hydralazine, 100 mg three times a day, propranolol, 160 mg twice a day, and chlorothiazide, 500 mg/day, for hypertension suddenly developed rapidly expanding ulcers that looked like pyoderma gangrenosum. Arthralgias, fevers, and occasional shortness of breath were also noted. A pericardial effusion was diagnosed by echocardiography. The antinuclear antibody (ANA) titer on routine mouse liver substrate was initially negative, but the ANA titer was positive (1:1,920) on human epithelioid cell substrate. Antibodies to histones and single-stranded DNA were also elevated. After discontinuing hydralazine, all signs and symptoms cleared over a 4-week period. At the time of discharge the ANA titer had decreased to 1:480.

Antibodies, Antinuclear↗

Malignant pyoderma.

Malignant pyoderma is a destructive, ulcerating skin disease that occurs chiefly in young adults. Only eight cases of this rare disease have been reported. The head and neck have been involved in all cases, and a predilection for the periauricular region has been noted in several of the cases. Although the disease is progressive and chronic, responses to high-dose systemic corticosteroids have been noted, but usually a flare is associated with a reduction in dose. An additional case is described in which therapy with isotretinoin and sulfapyridine led to complete remission.

Adolescent↗

Chlorambucil--an effective corticosteroid-sparing therapy for pyoderma gangrenosum.

A 21-year-old man, with a past history of severe aphthosis and arthritis between age 5 and 15 years, presented at age 18 years with a large leg ulceration, which developed after minor trauma. At that time he was otherwise healthy and taking no medications. Between 1983 and 1986 the patient failed to respond to intensive topical care to the ulcer, two attempted skin grafts, oral prednisone (up to 200 mg/day), sulfasalazine, dapsone (200 mg/day), multiple trials of intralesional triamcinolone acetonide, hyperbaric oxygen, azathioprine, methotrexate, clofazimine, minocycline, and three courses of pulse methylprednisolone therapy. Therapy with chlorambucil (4 mg/day) resulted in progressive improvement and complete healing with eventual cessation of all other therapy. The use of chlorambucil in pyoderma gangrenosum may be an effective adjunctive steroid-sparing therapy.

Administration, Oral↗

Pyoderma gangrenosum: classification and management.

Pyoderma gangrenosum (PG) has four distinctive clinical and histologic variants. Some have morphologic and histologic overlapping features with other reactive neutrophilic skin conditions. PG often occurs in association with a systemic disease, and the specific clinical features of the skin lesion may provide a clue to the associated disease. Management of PG depends on its type and severity and usually requires aggressive local and systemic treatment.

Humans↗

Chlorambucil is an effective corticosteroid-sparing agent for recalcitrant pyoderma gangrenosum.

BACKGROUND: Pyoderma gangrenosum (PG) may fail to respond to corticosteroids. Immunosuppressive and cytotoxic agents are useful in patients with recalcitrant disease. We describe our experiences with chlorambucil for PG. OBJECTIVE: Our purpose was to evaluate the effectiveness of oral chlorambucil in patients with PG recalcitrant to treatment with prednisone, immunosuppressive therapy, or both. METHODS: Six patients with recalcitrant PG were given oral chlorambucil 2 to 4 mg/day. Four patients were treated with a combination of prednisone and chlorambucil, and two received chlorambucil alone. Response was based on (1) a diminution in the size of the ulcers, or their complete healing, or (2) a decrease in the dose of corticosteroid therapy. RESULTS: Beneficial effects were noted within 6 to 8 weeks in all six patients, and corticosteroids were eventually discontinued in all patients. Currently only two patients are still receiving chlorambucil; the other four stopped chlorambucil after 6 to 24 months of treatment. Their disease has remained in remission for 4 to 9 years. Relapse of disease occurred within 1 to 4 months after stopping therapy in one of the two remaining patients or reducing the dose in the other. In both patients, the disease is again responding to treatment. Minimal chlorambucil toxicity has been noted, consisting of leukopenia in one patient. CONCLUSION: Our findings suggest that chlorambucil is an effective corticosteroid-sparing agent for the control of PG.

Adult↗

[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↗