Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Pyoderma Gangrenosum”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 1,117 records · Page 62Linked to original sources
PubMed2005

[Immunological abnormalities in myelodysplastic syndromes. Prospective study (series of 40 patients)].

UNLABELLED: The association of myelodysplastic syndromes (MDS) with auto-immune diseases and humoral disorders have already been reported. In this prospective study we tried to estimate type and the frequency of immunological associated diseases among patients affected by MDS. PATIENTS-METHODS: In this prospective study, auto-immune disease and humoral immunity disorders were systematically searched during MDS and conversely MDS searched during cytopenia. All MDS secondary to chemotherapy and the children's MDS were excluded. The MDS diagnosis was established according to FAB criteria and patients were classified in two groups A or B according to presence (group A) or not (group B) of dysimmune manifestations. RESULTS: Forty patients(19 males and 21 females, mean age of 56,6 years) with MDS have been enrolled during this period (group A: 20 patients). Associated diseases are following: systemic lupus erythematosus (three), lupus-like syndrome(one), sarcoidisis(one), Sjogrën syndrome(one), polyarthritis (two), chronic liver diseases (three), autoimmune thyroid diseases (two), pyoderma gangrenosum (one), Crohn's disease(one), haemolytic anaemia (one), and pericardial effusion(one). CONCLUSION: A wide spectrum of auto-immune manifestations is frequently reported in myelodysplastic syndromes. Further studies are necessary for discuss the current physiopathological hypothesis with their therapeutic relevance.

Adult↗
PubMed2000

The spectrum of skin disorders in abdominal stoma patients.

BACKGROUND: Skin integrity is essential for the normal usage of a stoma appliance. However, there is little published on the prevalence, nature or management of stoma-related skin disorders. Objectives To document stoma-related skin disorders in a large cohort of patients. METHODS: We sent a postal questionnaire to all surviving patients who had had abdominal stoma surgery at Hope Hospital, Salford, U.K. in the 10 years from 1 January 1989. Those reporting skin disease were invited to attend a clinic run by a dermatologist and a stoma-care specialist nurse. All lesions were categorized and swabs taken for microbiological examination. RESULTS: Of 525 surviving patients, 325 (62%) replied to the questionnaire. Of these, 73% reported a skin problem that had affected normal stoma bag use. Dermatoses included irritant reactions, particularly from leakage of urine or faeces (42%); pre-existing skin diseases, principally psoriasis, seborrhoeic dermatitis and eczema (20%); infections (6%); allergic contact dermatitis (0.7%) and pyoderma gangrenosum (0.6% annual incidence). A further 15% of patients with skin problems had persistent or recurrent dermatitis not explained by allergy, frank infection or faecal irritation. This responded to short-term treatment with topical corticosteroids. Further investigation is under way into its pathogenesis. CONCLUSIONS: Skin disorders are common in stoma patients, and various patterns can be recognized and effectively treated.

Abdomen↗
PubMed2005

Important cutaneous manifestations of inflammatory bowel disease.

Inflammatory bowel disease (IBD) has many extraintestinal manifestations. Cutaneous manifestations are usually related to the activity of the bowel disease but may have an independent course. Anyone presenting with IBD should be examined for cutaneous manifestations. Pyoderma gangrenosum is a severe painful ulcerating disease that requires moist wound management and, in the absence of secondary infection, systemic corticosteroids, cyclosporine, or both. Infliximab may also be used. Erythema nodosum is a common cause of tender red nodules of the shins. Management includes leg elevation, NSAIDs, and potassium iodide. Oral manifestations of IBD include aphthous stomatitis, mucosal nodularity (cobblestoning), and pyostomatitis vegetans. Treatment should be directed both at the cutaneous lesions and at the underlying systemic condition.

Erythema Nodosum↗
PubMed2002

[Cutaneous complications in idiopathic inflammatory bowel disease].

A 29-year-old male patient with the anamnesis of inflammatory bowel disease and Grave-Basedowov disease was hospitalized because of rapidly spreading skin defect with affected muscle on the left shin. This skin defect appeared after the significant decreasing of corticoids. The small skin trauma preceded the pyoderma gangrenosum. First the skin disease was not right diagnosed and patient was cured by the excision of the defect. It caused tissue disintegration, muscle necrosis and extension of the defect. The whole leg was endangered. Patient was cured with corticoids and cyclosporin A after the right diagnosis. The defect healed and laboratory inflammatory markers decreased. The immunosuppresive therapy was changed to azathioprin, the corticoid therapy was interrupted. After three months the defect was healed.

Adult↗
PubMed2003

Preparing the wound bed 2003: focus on infection and inflammation.

Wound bed preparation is the promotion of wound closure through diagnosis of the cause, attention to patient-centered concerns, and correction of systemic and local factors that may delay healing. To enhance the evidence base that may be lacking, a review of relevant literature was conducted and combined with input from the International Wound Bed Preparation Advisory Board and the Canadian Chronic Wound Advisory Board to create an updated examination of practices. A template based on expert opinion of the clinical actions corresponding to each step in the paradigm of preparing the wound bed is presented and the effects of local factors (tissue debridement, infection or inflammation, moisture balance, and edge effect [TIME]) are discussed. This review differentiates increased bacterial burden/infection in the superficial and deep wound bed compartments from inflammation and provides a topical approach to treatment. Inflammatory conditions causing leg ulcers, including pyoderma gangrenosum and vasculitis are reviewed. The topical combination of silver with absorptive dressings has led to new therapeutic options for increased bacterial burden in the surface wound compartment. A compilation of the available systematic reviews for the treatment of infection has been included as a background for the expert opinion.

Acute Disease↗
PubMed1994

A case of the 'dapsone syndrome'.

The 'dapsone syndrome' developed in a 44-year-old woman who was treated for pyoderma gangrenosum with 100 mg/day dapsone for about 5 weeks. Symptoms included fever, malaise, jaundice with hepatic dysfunction, lymphadenopathy, mononucleosis and dermatitis. These symptoms disappeared with 30 mg/day of oral prednisolone. A lymphocyte stimulation test with dapsone was positive, as was the delayed-type intradermal skin test with 0.5 and 0.05% dapsone in saline. Immunohistochemical studies of the rash and skin test reaction revealed that the dominant infiltrating T cells in the upper dermis were of the Leu 2a+ cytotoxic/suppressor-type rather than the Leu 3a+ helper/delayed hypersensitivity-type.

Adult↗
PubMed2006

Familial Mediterranean fever and the other autoinflammatory syndromes: evaluation of the patient with recurrent fever.

PURPOSE OF REVIEW: The aim of this article is to summarize recent clinical, genetic and pathophysiologic findings of familial Mediterranean fever and several of the other systemic autoinflammatory diseases, a recently recognized group of disorders characterized by seemingly unprovoked inflammation but lacking high-titer autoantibodies. Genetic and clinical tools are improving the ability of the clinician to better approach patients with periodic fever and inflammation. RECENT FINDINGS: The spectrum of reported genetic mutations and susceptible ethnicities for the hereditary periodic fever subset of the autoinflammatory diseases has continued to expand. At the same time, the pathogeneses of many of these diseases are now understood to involve different aspects of a common pathway, largely affecting inflammatory cascades related to IL-1 or tumor necrosis factor-alpha. Three of these diseases which have been grouped as the cryopyrin-associated periodic syndromes result from defects in the same gene, and all three appear to respond well to anti-IL-1 therapy although controlled trials are still in progress. In addition, cytokine-based therapies are also now under investigation for hyperimmunoglobulinemia D with periodic fever syndrome and pyogenic sterile arthritis, pyoderma gangrenosum, and acne syndrome. SUMMARY: The identification of the genes and proteins mutated in many of the autoinflammatory diseases has broadened our understanding of the regulation of inflammation and the immune system, and provided the basis for the use of targeted therapies in these syndromes. We propose an algorithm for the evaluation of a patient with periodic fever, taking into account the patient's age, ethnicity, symptoms and signs, and results from laboratory and genetic testing.

Acne Vulgaris↗
PubMed1995

Unusual cutaneous manifestations of myelodysplastic syndrome.

We report a 58-year-old patient with acute myeloid leukaemia who developed an oedematous erythema resembling Sweet's syndrome, accompanied by atypical erythema nodosum and bullous pyoderma gangrenosum. Examination of skin biopsies showed dense infiltration with mature neutrophils, although there was peripheral blood leucocytopenia. The oedematous erythema worsened after he was treated with granulocyte-colony-stimulating factor (G-CSF), which was given for his leucocytopenia. We suggest that when a neutrophilic dermatosis complicates leukaemia, alternatives to G-CSF should be considered for the treatment of leucocytopenia. Corticosteroids were very effective in controlling the skin lesions in our patient.

Acute Disease↗
PubMed2005

Extraintestinal manifestations of Crohn's disease.

In each case of extraintestinal manifestations of Crohn's disease, active disease, if present, should be treated to induce remission, which may positively influence the course of most concomitant extraintestinal manifestations. For some extraintestinal manifestations, however, a specific treatment should be introduced. This latter part of disease management will be discussed in this chapter, in particular for pyoderma gangrenosum, uveitis, spondylarthropathy--axial arthropathy--and primarysclerosing cholangitis, which have also been described in quiescent Crohn's disease. Few new drugs for the treatment of extraintestinal manifestations of Crohn's disease have been developed in the past and only the role of infliximab has increased in Crohn's disease-related extraintestinal manifestations. Drugs specifically aimed at this treatment, stemming from a few randomized controlled studies or case series, are sulfasalazine, 5-ASA, corticosteroids, azathioprine or 6-mercaptopurine, methotrexate, infliximab, dapsone and cyclosporine or tacrolimus.

Cholangitis, Sclerosing↗
PubMed2004

Infevers: an evolving mutation database for auto-inflammatory syndromes.

The Infevers database (http://fmf.igh.cnrs.fr/infevers/) was established in 2002 to provide investigators with access to a central source of information about all sequence variants associated with periodic fevers: Familial Mediterranean fever (FMF), TNF Receptor Associated Periodic Syndrome (TRAPS), Hyper IgD Syndrome (HIDS), Familial Cold Autoinflammatory Syndrome/Muckle-Wells Syndrome/Chronic Infantile Neurological Cutaneous and Articular Syndrome (FCAS/MWS/CINCA). The prototype of this group of disorders is FMF, a recessive disease characterized by recurrent bouts of unexplained inflammation. FMF is the pivotal member of an expanding family of autoinflammatory disorders, a new term coined to describe illnesses resulting from a defect of the innate immune response. Therefore, we decided to extend the Infevers database to genes connected with autoinflammatory diseases. We present here the biological content of the Infevers database, including the introduction of two new entries: Crohn/Blau and Pyogenic sterile arthritis, pyoderma gangrenosum and acne (PAPA syndrome). Infevers has a range of query capabilities, allowing for simple or complex interrogation of the database. Currently, the database contains 291 sequence variants in related genes (MEFV, TNFRSF1A, MVK, CARD15, PSTPIP1, and CIAS1), consisting of published data and personal communications, which has revealed or refined the preferential mutational sites for each gene. This database will continue to evolve in its content and to improve in its presentation.

Arthritis↗
PubMed1995

Squamous syringometaplasia in association with annular elastolytic granuloma.

Squamous syringometaplasia, squamous metaplasia of eccrine glands, is considered to be an important component of pseudoepitheliomatous hyperplasia, which can be a response to a variety of conditions. Recently, squamous syringometaplasia was described as occurring in lobular panniculitis, pancreatic panniculitis, and pyoderma gangrenosum. We report a case of annular elastolytic granuloma with associated squamous syringometaplasia. The initial biopsies were superficial, and the squamous syringometaplasia extended to the base of the specimen, closely resembling squamous cell carcinoma. Immunohistochemical stains for carcinoembryonic antigen outlined eccrine structures. The special stains and an excisional biopsy helped confirm the diagnosis. In addition, the clinical course has been consistent with the diagnosis of annular elastolytic granuloma. It is postulated that the dense granulomatous inflammatory infiltrate in annular elastolytic granuloma damages the eccrine structures, and this damage initiates the process.

Antiporters↗
PubMedDate not supplied

Leg ulcers: uncommon presentations.

Uncommon presentations like vasculitis or other immunologic causes and malignancy account for about 1% to 2% of patients suffering from leg ulcers. We focus on such uncommon leg ulcers including: cutaneous vasculitis causing cutaneous ulceration, other immunologic or metabolic cutaneous lesions such as pyoderma gangrenosum and necrobiosis lipoidica, and ulcers based on neoplastic etiology. A short description on leg ulcers in the tropics is also included. The described uncommon presentations of leg ulcers are typically difficult to diagnose and treat; it is a specialist's job to take care of patients with these types of ulcers. Multidisciplinary specialized wound healing concepts integrated in the national health care system, as an accepted expert function, is the ideal way to organize the wound healing area. Such a system would result in an earlier diagnosis and more sufficient treatment for patients with uncommon presentations of leg ulcers.

Adult↗
PubMed2006

The systemic autoinflammatory diseases: inborn errors of the innate immune system.

The autoinflammatory syndromes are a newly recognized group of immune disorders that lack the high titers of self-reactive antibodies and T cells characteristic of classic autoimmune disease. Nevertheless, patients with these illnesses experience unprovoked inflammatory disease in the absence of underlying infection. Here we discuss recent advances in eight Mendelian autoinflammatory diseases. The causative genes and the proteins they encode play a critical role in the regulation of innate immunity. Both pyrin and cryopyrin, the proteins mutated in familial Mediterranean fever and the cryopyrinopathies, respectively, are involved in regulation of the proinflammatory cytokine, IL-1beta, and may influence the activity of the transcription factor, NFkappaB. NOD2, the Blau syndrome protein, shares certain domains with cryopyrin and appears to be a sensor of intracellular bacteria. PSTPIP1, mutated in the syndrome of pyogenic arthritis with pyoderma gangrenosum and acne, interacts both with pyrin and a protein tyrosine phosphatase to regulate innate and adaptive immune responses. Somewhat unexpectedly, mutations in the p55 TNF receptor lead not to immunodeficiency but to dramatic inflammatory disease, the mechanisms of which are still under investigation. Finally, the discovery of the genetic basis of the hyperimmunoglobulinemia D with periodic fever syndrome has provided a fascinating but incompletely understood link between cholesterol biosynthesis and autoinflammation. In this manuscript, we summarize the current state of the art with regard to the diagnosis, pathogenesis, and treatment of these inborn errors of the innate immune system.

Adaptor Proteins, Signal Transducing↗
PubMed2001

Clinical use of Infliximab in Crohn's disease: the Edinburgh experience.

UNLABELLED: Infliximab is an established treatment for steroid-resistant and fistulating Crohn's disease. Although efficacy has been shown in clinical trials, financial implications often limit its use and limited data exist regarding clinical practice. AIMS: To audit the clinical effectiveness of Infliximab. METHODS: We prospectively audited 50 consecutive patients [28 females; median age, 34 years (17-70 years)]. Disease activity and response rates were assessed by the Harvey-Bradshaw index. Clinical and disease data were collected and blood was taken for inflammatory markers, complement and double-stranded DNA antibodies. Patients received Infliximab at 5 mg/kg and were followed for 12 weeks. RESULTS: Indications for Infliximab were refractory Crohn's disease in 39 patients, fistulating Crohn's disease in six, pyoderma gangrenosum in one, pouchitis in two and coeliac disease in two. Thirty-one (79%) of the refractory Crohn's disease patients and four (66%) of the fistulating patients responded at 4 weeks. Twenty-one (54%) of the refractory Crohn's disease patients had a continued response at 12 weeks. Perianal disease was more prevalent in non-responders (7/8 vs. 12/31, P < 0.02). CONCLUSIONS: Response rates to Infliximab in our group are comparable to those of clinical trials. Despite the expense, it remains a useful adjunct to treatment in this otherwise difficult group of patients. Patients with perianal disease responded less well in our cohort.

Adolescent↗
PubMed2001

[Minocycline].

Minocycline belongs to the second generation class of cyclines. It was synthesized in 1967 and marketed in 1972. Minocycline has an antiinfectious activity with a spectrum similar to that of other cyclines, notably against Chlamydias, Treonema and Proprionibacterium acenes. The antiinflammatory activity is associated with this antiinfectious action is greater than that of first generation cyclines with specifically a modulator effect on epidermal cytokines. The pharmokinetics of minocycline is characterized by an excellent absorption, a long half-life and an important lipophilic property inducing good tissue distribution. Clinical trials of minocycline have mainly been performed in sexually transmissible diseases and in acne, a field where randomized studies are the most frequent. These trials show that the effect of minocycline is not stronger than first generation cyclines or doxycycline, but that the action is quicker than that of tetracycline at the dose of 500 mg a day. Minocycline is also efficient in nocardiasis, mycobacteriosis, leprosy, Lyme disease, pyoderma gangrenosum, autoimmune bullous dermatitis, Carteaud disease, and prurigo. However, the effect of minocycline in these different conditions has always been evaluated in open trials with a small number of patients. The usual side effects of cyclines, i.e. digestive problems, fungal infections, are less frequent than with first generation cyclines. No photosensitivity has been demonstrated although pigmentations have been described. Dizziness is a specific side effect of minocycline. Furthermore, rare but severe side effects have been reported, including hypersensitivity syndrome, autoimmune hepatitis, and lupus. Regular indications for minocycline in dermatology are acne and three sexually transmissible diseases (mycoplasm, chlamydia, treponema). Proposed dosage is 100 mg per day in sexually transmissible disease with a reduction to 50 mg per day after 15 days in acne.

Acne Vulgaris↗
PubMedDate not supplied

Suspected white-tail spider bite and necrotic ulcers.

AIM: To describe the clinical features, investigation, diagnosis and treatment of ulcers attributed to white-tail (WT) spider bites or necrotic arachnidism. METHODS: The study was a prospective case series of patients referred to the Hunter Area Toxicology Service (a tertiary referral toxicology unit servicing a population of 500 000) with an ulcer or skin lesion that had been attributed to either a suspected WT spider bite or necrotic arachnidism. Eleven patients with skin lesions or necrotic ulcers were referred between January 2000 and June 2002. RESULTS: In two patients that were inpatients in other hospitals, investigation and follow up was not possible. In both cases there was no history of spider bite and Staphylococcus aureus was cultured. In nine patients, a diagnosis other than spider bite was made following appropriate investigation and follow up, including: (i) two cases of dermatophytoses, (ii) three staphylococcal infections, (iii) one case of pyoderma gangrenosum, (iv) one case of cutaneous polyarteritis nodosa, (v) one case of Nocardia braziliensis and (vi) one infected diabetic ulcer. There was only one case where the person recalled seeing a spider bite them, but the patient did not collect the spider for identification. The median time to diagnosis was 3 weeks (interquartile range: 3-9 weeks) and 3.5 years in one case. Appropriate treatment was initiated once the correct diagnosis was made and all cases resolved. CONCLUSIONS: In this series, all cases initially referred as WT spider bites or necrotic arachnidism were found to have alternative diagnoses with appropriate investigations. This demonstrates that spider bites are an unlikely cause of necrotic ulcers and that all ulcers should be properly investigated with bacterial, fungal and mycobacterial cultures and skin biopsy for histopathology.

Adult↗
PubMed2006

From acute febrile neutrophilic dermatosis to neutrophilic disease: forty years of clinical research.

In 1964, Sweet described an acute febrile neutrophilic dermatosis. It is now widely accepted that Sweet's syndrome belongs to a group of associated neutrophilic dermatoses. Although clinically dissimilar, Sweet's syndrome, pyoderma gangrenosum, subcorneal pustular dermatosis, erythema elevatum diutinum, and a few other conditions can be considered a part of this same pathologic spectrum of inflammatory disorders because of (1) the existence of transitional and overlap forms; (2) the similar histopathologic feature of an infiltrate by normal polymorphonuclear leukocytes; (3) the possible occurrence of extracutaneous neutrophilic infiltrates, defining the neutrophilic disease; and (4) the frequent association with systemic diseases. According to the localization of the neutrophilic infiltrate, we describe neutrophilic dermatoses en plaques (dermal), superficial (epidermal), and deep (dermal and hypodermal). Almost every organ of the body may be involved by a neutrophilic aseptic inflammation. The main systemic diseases associated with neutrophlic dermatoses are hematologic, gastrointestinal, and rheumatologic diseases. Although the pathophysiology of these conditions is still poorly understood, treatment with systemic anti-inflammatory agents is usually efficacious.

Abscess↗
PubMed2004

Rheumatoid arthritis-associated inflammatory leg ulcers: a new treatment for recalcitrant wounds.

Patients with rheumatoid arthritis may develop leg ulcers of varied aetiologies, including venous disease, infection and inflammation (vasculitis or pyoderma gangrenosum). The leg ulcers in rheumatoid arthritis patients may involve several of these aetiological factors and are often difficult to heal. Both the ulcers and the treatments are often painful, and these ulcers may be present for years. A new oxidised regenerated cellulose and collagen dressing has been developed for slow-to-heal wounds and may have a role in the management of superficial inflammation that may persist in many of these ulcers, although clinically it is difficult to distinguish this from critical colonisation or frank infection. Venous disease requires compression therapy. Deep compartment infection should be treated with systemic antimicrobials, and inflammatory processes extending beyond the superficial wound base require disease-specific systemic anti-inflammatory agents. Four patients with recalcitrant wounds resistant to best practice were treated successfully with this new dressing combined with a strategy to control bacterial burden.

Aged↗