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Treatment of pyoderma gangrenosum with cyclosporine.

BACKGROUND AND DESIGN: Pyoderma gangrenosum is a chronic inflammatory ulcerative skin disease of unknown origin, often associated with various diseases including inflammatory bowel disease, inflammatory arthritis, monoclonal gammopathies, hepatitis, and myeloproliferative disorders. Treatment of associated systemic disorders may improve the ulcers, but lesions may be recalcitrant and persist for months to years. Therapy for pyoderma gangrenosum includes high-dose systemic corticosteroids, sulfa drugs such as sulfasalazine, clofazimine, and immunosuppressive agents such as mercaptopurine and azathioprine; these drugs are sometimes ineffective. RESULTS: We present a series of 11 patients with pyoderma gangrenosum, with a wide range of underlying diseases, whose ulcers were refractory to usual therapy and who were treated with low-dose cyclosporine. Ten of the 11 patients cleared rapidly and completely with cyclosporine therapy. CONCLUSIONS: Cyclosporine should be seriously considered as a primary form of treatment for pyoderma gangrenosum.

Adult↗

Subcorneal pustulosis and Pyoderma gangrenosum associated with a biclonal gammopathy.

Pyoderma gangrenosum and subcorneal pustulosis are two neutrophilic dermatoses. Their occurrence in the same patient is rare and may be related to an IgA dysglobulinemia. We report a case presenting these two conditions associated with a biclonal benign IgA and IgG gammopathy. A 67-year-old man exhibited typical pyoderma gangrenosum associated after three years duration with subcorneal pustulosis lesions, confirmed by cutaneous biopsy. Laboratory results showed a biclonal benign IgA and IgG kappa gammopathy. Therapeutic management was difficult: Pyoderma gangrenosum responded well to corticosteroids but subcorneal pustulosis management was harder and treatments were poorly effective.Pyoderma gangrenosum and subcorneal pustulosis are a part of the neutrophilic spectrum. Their association has been only reported in eleven patients. In eight cases, an IgA dysglobulinemia was associated suggesting its responsibility in the occurrence of both dermatoses. Treatments are various and not fully effective. If the Pyoderma gangrenosum usually responds to corticosteroids, the subcorneal pustulosis treatments are not well defined and often not efficient. Our case illustrates the dissociated evolution of these two dermatoses and their difficult global management. During the follow-up, a regular search for dysglobulinemia is required in order to detect malignant transformations.

Aged↗

[Pyoderma vegetans as an early sign of HIV infection].

Skin and mucosal lesions are described in a 40-year-old man who suffered from HIV infection in the stage of secondary diseases. The diagnosis was supported by demonstration of antibodies to HIV in the immune blotting test. The clinical manifestations were reduced to steady fever, generalized lymphadenopathy, pyoderma vegetans and chancriform pyoderma, candidiasis of the oral cavity, and seborrheic dermatitis, HIV infection was diagnosed 4 years after the appearance of pyoderma which initially was amenable by external antibacterial agents and then became resistant to them. Eruptions of pyoderma vegetans simulating wide condylomas were located in inguinal folds, on the internal surface of the thighs and scrotum; the chancriform focus was located in the internal preputial layer. The treatment with azothymidine combined with antibacterial and antimycotic agents led to the improvement of the patient's general status and to the reduction of local lesions. However, on recommendations of an unknown witch doctor who started treating him with the aid of reflexotherapy, phytotherapy and bioenergetic actions the patient stopped receiving the treatment prescribed and did not report for control examinations. After 5 months he died of brain edema.

Acute Disease↗

[The immunocorrective therapy of pyoderma caused by staphylococci multiply resistant to antibiotics].

Examinations of 126 patients with staphylococcal pyodermas have helped detect a relationship between the total antibiotic sensitivity of staphylococci isolated from the involved skin and the patient's immunity status. The total antibiotic sensitivity of staphylococci in directly proportional to the blood level of T-lymphocytes, to the degree of their sensitization to staphylococcus in the leukocyte migration inhibition test, and to the value of IgM/IgG ratio. A new synthetic preparation of thymus, thymogen, had a modulating effect on the lymphocytes of pyoderma patients, manifesting by the increment of the total count of T-lymphocytes and T-helpers. A reduction of the total antibiotic resistance of the agent in foci of skin infection and normalization of the immunologic reactivity parameters were recorded in 23 patients with chronic pyodermas after a course of thymogen therapy. An effective method for the management of chronic pyoderma by a combination of thymogen with antibiotics is suggested.

Adjuvants, Immunologic↗

Present status of pyoderma gangrenosum. Review of 21 cases.

This article summarizes the management of 22 cases of pyoderma gangrenosum over the past four years at the hospital of the University of Pennsylvania, Philadelphia. Eighteen patients with pyoderma gangrenosum were studied using the most sensitive routine laboratory method for detection of monoclonal immunoglobulins, immunofixation electrophoresis. Four cases of IgA gammopathy were detected, confirming previous reports of the incidence of monoclonal gammopathy in pyoderma gangrenosum. High-dose glucocorticoid therapy (pulse therapy) is an effective treatment for some severe, refractory cases of pyoderma gangrenosum. Eight patients were treated with pulse therapy. Six responded favorably, and none had serious complications.

Adrenal Cortex Hormones↗

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↗

Epidemiology of streptococcal pyoderma in an orphanage community of a tropical country.

A group of 89 children residing in an orphanage, situated about 12 km from Vellore town, Tamil Nadu, India, were surveyed for streptococcal pyoderma, every month, from October 1976 through to December 1978. The prevalence rate of pyoderma as defined by the isolation of group A streptococci (GAS) was 10.1% with monthly variations from 2.1% to 17.1%. Three meteorological data, namely, temperature, humidity and rainfall did not show any significant relationship with seasonal patterns of pyoderma. However, a hot and humid climate, prevalent for most of the year in Vellore, may be linked to the endemicity of this condition. Studies performed on a limited number of GAS strains showed a variety of M types in the lesions. During this survey, poststreptococcal glomerulonephritis (PSGN) was not detected. In one child, a moderate albuminuria was observed; this resolved subsequently. We attest that an active surveillance of detection and treatment of pyoderma in a highly susceptible population plays an important role in the prevention of PSGN.

Adolescent↗

Pyoderma gangrenosum or cutaneous lymphoma: a difficult clinical diagnosis.

Pyoderma gangrenosum is frequently associated with an underlying condition such as ulcerative colitis or a myeloproliferative syndrome. However, lymphoproliferative malignancies have only rarely been seen concurrently with pyoderma gangrenosum. We present the case of a patient with small lymphocytic lymphoma who noted a slowly enlarging skin ulcer that was clinically consistent with pyoderma gangrenosum. Examination of a biopsy specimen showed infiltration of the skin with lymphoma cells as well as deeper necrotic material and thrombosis of vessels that were infiltrated by lymphoma. This case illustrates the difficulty of differentiating pyoderma gangrenosum from cutaneous lymphoma clinically.

Diagnosis, Differential↗

Blastomycosis-like pyoderma.

Seven patients with blastomycosis-like pyoderma had skin lesions of four months' to six years' duration. The criteria for the diagnosis of blastomycosis-like pyoderma include the clinical presentation of large verrucous plaques with multiple pustules and elevated border, pseudoepitheliomatous hyperplasia with abscess histologically, and the growth of at least one pathogenic bacteria from the culture of a tissue-biopsy specimen. The differential diagnosis includes deep fungus infection (especially North American blastomycosis), bromoderma, pyoderma gangrenosum, mycobacterial infections, giant keratoacanthoma, and squamous cell carcinoma. Generally, the patients had one or more conditions that could have affected their systemic or local immunologic competence to infection. We believe that the clinical and histologic features in these cases of blastomycosis-like pyoderma were produced by an unusual, exaggerated, vegetating-tissue reaction to a primary or secondary bacterial infection.

Adult↗

[Pyoderma gangrenosum and haemopathy. Report of three cases (author's transl)].

The authors report three personal cases of phagedenic pyoderma associated with hemopathy. Studying twenty-five other cases, described in the reviews, the question can be debated on three levels: 1) The clinical characteristics of hemopathic pyoderma remain non-specific. However blisters, pustules, and even vegetating lesions occur very often; 2) the etiology of hemopathy is subject to change; acute leukaemia or myeloproliferation syndrome. During the polyglobulars associated with pyoderma, anemia and myelofibrosis appear quite constantly; 3) finally on the pathogenic level, the recent works tend towards the hypothesis of a damage in the functioning of the polymorphonuclears hence displaying the increase in the inflammation during phagedenic pyoderma.

Adolescent↗

[Pyoderma gangrenosum and biclonal gammapathy probably due to multiple myeloma (author's transl)].

The authors report the observation of pyoderma gangrenosum (P. G.) leading to the discovery of an underlying biclonal gammapathy, which despite the absence of bony lesions, almost certainly represents a malignant myeloma. The authors have reviewed the different known associations with pyoderma gangrenosum, with special reference to benign and malignant gammapathies. Rare cases of biclonal gammapathies with pyoderma gangrenosum have been published but none of a malignant nature. This therefore appears to be the first reported cases. The authors are aware of the reported tendency of pyoderma gangrenosum lesions to appear in areas of previous trauma, as may be the case in this patient. Finally systemic corticosteroid therapy produced a rapid remission of the skin lesions, but with the reactivation of known treated pulmonary tuberculosis.

Gangrene↗

[Aseptic adenitis in pyoderma gangrenosum].

INTRODUCTION: Extra-cutaneous manifestations of neutrophilic dermatosis are rare. Symptomatic cases are usually multiform and confusing. We report a case with an aseptic lymph node abscess associated with pyoderma gangrenosum. CASE REPORT: A 28-year-old woman with past history of pyoderma gangrenosum was seen for abdominal symptoms and fever related to an epigastric mass. Due to signs indicating abscess formation, surgery was performed and led to total regression of the symptomatology. Recurrence was evidenced 3 months later with skin lesions which were negative on bacteriological examinations. The diagnosis of lymph node manifestations of pyoderma gangrenosum was retained. General corticotherapy was very effective. Relapse occurred again 6 years later and was treated with thalidomide. DISCUSSION: Lymph node involvement has been described in Sneddon-Wilkinson's disease associated with pyoderma gangrenosum. In neutrophilic dermatosis, pulmonary manifestations appear to be the least exceptional of the extra-cutaneous lesions. The pathophysiology has not been elucidated although recent hypotheses suggest that G-CSF could be involved. Corticotherapy is remarkably effective in most cases but thalidomide could be an interesting alternative treatment.

Abdominal Pain↗

[Ulcerative colitis associated with pyoderma gangrenosum].

The authors present a 42-year-old man's case in whom the onset of chronic ulcerative colitis and pyoderma gangrenosum was simultaneous. Pyoderma gangrenosum began as bullae on his chest and lower limb. The main symptoms were fever, anaemia, hypoproteinaemia beyond the skin lesions, and the abdominal symptoms characterizing chronic ulcerative colitis were absent. The thorough internal check-up and the micribiologic and histologic examinations of the skin disorders made clear that the lesions mentioned were the skin symptoms of a systemic disease. Sulfasalazin and prednisolone therapy of the underlying disease and the local non-specific treatment of the cutaneous lesions resulted together in the patient's rapid improvement. The authors give a brief review of pyoderma gangrenosum and of the connection between pyoderma gangrenosum and chronic ulcerative colitis.

Adult↗

Treatment of pyoderma gangrenosum with methotrexate.

Pyoderma gangrenosum is an ulcerative skin condition of unknown cause. It is often refractory to treatment, requiring high dosages of immunosuppressive medications. A patient with idiopathic pyoderma gangrenosum was successfully treated with methotrexate and weaned off steroids for the first time in four years. The patient had received multiple skin grafts and failed topical treatment, rifampin, dapsone, azathioprine, and intralesional steroids. She required prednisone 60 mg/day for lesion healing. Methotrexate was given orally, with rapid response, allowing discontinuation of prednisone seven months after beginning therapy. There is evidence of neutrophil dysregulation in pyoderma gangrenosum. Methotrexate has been shown to decrease neutrophil migration and chemotaxis, suggesting a role for its use in pyoderma gangrenosum.

Adult↗

Intravenous cyclophosphamide pulses in pyoderma gangrenosum: an open trial.

OBJECTIVE: To evaluate the potential efficacy of intravenous bolus cyclophosphamide (IVCY) in patients with pyoderma gangrenosum. METHODS: Consecutive patients with a diagnosis of pyoderma gangrenosum seen in a period of 3 years in tertiary care referral center were included. Patients received IVCY 500 mg/m2 of body surface area, every month until reaching a maximum of 6 doses, or healing of their ulcers or a lack of response after 3 doses. Patients were assessed every month during the time they received IVCY and every 3 months thereafter. The assessments included number and size of ulcers, and a safety profile of the study drug. Complete remission was defined as 100% ulcer healing, partial remission as a decrease > or = 50% but less than 100%, and therapeutical failure if the size of the ulcer increased or decreased < 50%. RESULTS: Nine patients were included, 6 were men, the mean age was 46 yrs (range 24-76). The mean disease duration was 3.3 yrs (range 1 week to 9 yrs). Four patients had idiopathic pyoderma gangrenosum, 3 had associated rheumatoid arthritis, and 2 had associated systemic lupus erythematosus. Complete remission was observed in 7 patients, partial in one, and failure in one. Relapses were observed, 3 months after the last IVCY (2 cases) and 12 months after the last IVCY (one case). Transitory thrombocytopenia and leukopenia developed in one patient and nausea and vomiting in another. CONCLUSION: IVCY appears effective in controlling the lesions of pyoderma gangrenosum and inducing remission for a substantial period in many individuals.

Adult↗

[Pristinamycin versus oxacillin in the treatment of superficial pyoderma. A multicenter randomized study in 293 outpatients].

BACKGROUND: Superficial pyoderma occurs frequently. Generally, the benign infection is caused by Staphylococcus aureus and/or a group A streptococci. The subject is controversial, but treatment usually is based on narrow-spectrum antibiotics active against both germs. PATIENTS AND METHODS: A multicentric, randomized, double-blind, double-placebo study was conducted to compare pristinamycin (1 g b.i.d.) with a reference antibiotic, oxacillin (1 g b.i.d.) for 10 days. Inclusion criteria were: both sexes, age 15-80 years, clinical diagnosis of superficial pyoderma (impetigo, wound infection within the last 15 days, furunculosis, carbuncle, perionyxis), informed consent. The general practitioner investigators (n = 52) were assisted by 9 dermatologist-coordinators. Clinical diagnosis was validated by a committee of experts at the end of the study after analyzes of the photos and bacteriological results obtained on samples taken at the practitioner's office on visit 1 (D0), visit 3 (D14 +/- 3) and visit 4 (D25 +/- 3). Successful treatment was defined by clinical, bacteriological and photographic efficacy at visit 3 (equivalence analysis: one-way 95 p. 100 confidence interval). RESULTS: There were 293 included patients given pristinamycin (n = 151) or oxacillin (n = 142). Mean age of analyzed patients was 40 +/- 17 years. Diagnosis was confirmed in 255 patients in accordance with the protocol: furunculosis or carbuncle (n = 100), recently superinfected wound (n = 97), impetigo (n = 41), acute perionyxis (n = 17). Thirty-five patients (12 p. 100) were considered to have been wrongly included. The germs most often isolated were: Staphylococcus aureus (n = 126), group A streptococci (n = 13), group B streptococci (n = 5) and P. multocida (n = 3). At visit 3, the two treatments were found to be equivalent with a success rate of 86.7 p. 100 for pristinamycin and 89.8 p. 100 for oxacillin (CI [*9.97]). Tolerance was statistically comparable between the two treatments (27 to 32 percent minor side effects). DISCUSSION: This study is the first performed in outpatients attended by general practitioners with diagnostic confirmation on both bacteriological and photographic evidence of superficial pyoderma. The results obtained demonstrate the good reliability of such studies although 12 p. 100 of the patients were wrongly included, a factor which should be taken into account for future studies. The efficacy and tolerance of pristinamycin were statistically equivalent to those of oxacillin for all the patients with superficial pyoderma. Nevertheless, the subgroup of patients with folliculitis gave rather heterogeneous bacteriology and therapeutic results.

Adolescent↗

The epidemiology and natural history of streptococcal pyoderma: an endemic disease of the rural southern United States.

In order to study the natural history of endemic pyoderma, the host and environmental risk factors to infection, the immunologic response and the risk of acute glomerulonephritis (AGN) a prospective study was done between June 29 and December 13, 1970 in 444 black children aged 2-6 years attending project Headstart centers in Holmes County, Mississippi. The weekly prevalence of pyoderma was about 40-50% during July and August but decreased to 4% during the last week of the study. "Pyoderma-type" serotypes of group A streptococci were isolated from about 70% of the skin lesions and similar serotypes were also commonly isolated from the pharynx. The seasonal prevalence and T and M typing pattern of most of the pharyngeal isolates mirrored the skin isolates. Many of the streptococci appear to belong to previously unrecognized M-types and one strain has been designated provisional M type 67 by the International Subcommittee on Pneumococci and Streptococci. Staphylococci were also isolated commonly from the skin lesions, especially late in their evolution. Despite an 80% incidence of streptococcal pyoderma during the summer months, only 3 children (0.67%) developed AGN; all of these children had clinically mild disease. The risk of a major outbreak of AGN in populations like these is substantial. Surveillance for clusters of AGN is indicated and widespread benzathine penicillin prophylaxis should be used in the event of an outbreak. Also, further research to determine the long term prognosis of clinically mild AGN and to detect useful laboratory markers of nephritogenicity are indicated.

Age Factors↗

Pyoderma gangrenosum after caesarean section: a case report.

BACKGROUND: Pyoderma gangrenosum is a rare ulcerative skin disease. The diagnosis is based on clinical features and excluding other causes of skin ulcers, as it does not have characteristic histopathology or laboratory findings. The etiology is poorly understood. Lesions can develop spontaneously, after surgery or after trauma. CASE PRESENTATION: We present the case of a 32-year-old woman with ulcerative wound defect after caesarean section. The wound was not healing despite standard wound care and antibiotic treatment. Pyoderma gangrenosum was diagnosed and after high dose corticosteroids wound healing started. CONCLUSION: Early diagnosis and subsequent treatment of pyoderma gangrenosum are crucial for limiting scar tissue. Diagnosis of pyoderma gangrenosum could easily be missed since gynaecologists are rarely confronted with this disorder.

Journal Article↗