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Crohn's disease; analysis of 105 patients.

In this paper, 105 patients with Crohn's disease, (47 M, 58 F), mean age 37.4 +/- 42 years were evaluated clinically, demographically and epidemiologically. Mean age of patients at the time of diagnosis was 26.5 +/- 10.9 years. Follow-up period was 2.7 +/- 2.1 years on average. On admission, symptoms or signs were as follows: right lower quadrant pain 90.5%, abdominal mass 18.1%, enterocutaneous fistula 11.4% and subileus 9.5%. Diagnosis of Crohn's disease was established during appendectomy in 14 patients (13.3%). Family history of inflammatory bowel disease was determined only in six patients (5.7%). Intestinal localization were as follows: ileo colonic 52%, ileal 38%, colonic 10%. Clinical forms were inflammatory (68%), fistulous (23%) and obstructive (9%). Sacroiliitis (7.6%), ankylosing spondylitis (4.7%), erythema nodosum (2.9%), pyoderma gangrenosum (1%) were detected as extraintestinal manifestations. Of the patients, 12.4% underwent surgical intervention due to abscess drainage in 6.6%, fistulectomy in 3.8%, stricture resection in 1.9%. Medical therapy alone was sufficient in 75.3% of patients. As a result, our cases mentioned in this paper reflect the general characteristics of Crohn's disease and prominence of regular visits and treatment.

Abdominal Pain↗

Peristomal skin complications: prevention and management.

Peristomal skin complications are the most common reason ostomy patients visit an outpatient wound, ostomy, and continence nursing service. Prevention and management of peristomal skin complications are critical components of ostomy care. Identifying risk factors for the occurrence of peristomal skin complications according to types of injury and clinical features can help optimize assessment and management approaches. Treatment can further be addressed based on etiology - chemical injury (irritant contact dermatitis, pseudoverrucous lesions, and encrustations); mechanical injury (pressure/shear, stripping, mucocutaneous separation, mucosal transplantation); infection (Candidiasis, folliculitis); immunologic disorders (allergic contact dermatitis); and disease-related lesions (varices, pyoderma gangrenosum, malignancy). The importance of prevention and the impact of having access to knowledgeable care providers cannot be over-emphasized.

Documentation↗

Skin disorders in association with monoclonal gammopathies.

Monoclonal gammopathy represents a condition characterized by clonal proliferation and accumulation of immunoglobulin producing B-cells. A variety of skin disorders are associated with an increased level of monoclonal immunoglobulin proteins. These skin disorders can be divided into two groups. The first group represents a direct consequence of plasma cell proliferation. The colonization of the plasma cell clone in the dermis expressed as a deposition of proteins related to the M component belongs to this group for which the pathogenesis is well identified, as is the case for example with AL amyloidosis and cryoglobulins. The second group represents skin disorders such as scleromyxedema and Schnitzler syndrome that are highly associated with an M component, or diseases such as pyoderma gangrenosum and leukocytoclastic vasculitis that are more weakly associated with increased levels of monoclonal immunoglobulins. In some other dermatoses such as pemphigus, bullous pemphigoid, epidermolysis bullosa aquisita, Sezary syndrome, lymphomatoid papulosis, urticaria pigmentosa, and acquired ichthyosis, only presumptions exist regarding associations with monoclonal gammopathies. In this the pathogenesis, therapy and prognosis of the most relevant dermatoses shall be described in order of their degree of association with monoclonal gammopathies, which shall also be discussed.

Humans↗

[Neutrophilic dermatosis in a patient with refractory anemia].

A 46-year-old man diagnosed as refractory anemia was hospitalized because of high fever and extensive erythema with ulceration in the femoral region. His peripheral blood examination showed marked leukocytosis (WBC 31,500/microliter:neutrophilic 90%) and anemia (Hb 8.6 g/dl. In spite of administration of antibiotics, the cutaneous ulcer rapidly extended to the right thigh and became necrotic. The bacterial culture of the cutaneous lesion showed no growth and a skin biopsy showed infiltration of neutrophils in the dermis. He became afebrile and his cutaneous lesion improved after administration of corticosteroid. When the dose of corticosteroid was decreased, cutaneous erythema and nodules appeared at other sites repeatedly, and disappeared after the dose of corticosteroid was increased. The cutaneous lesions had characteristics of both Sweet's syndrome and pyoderma gangrenosum. Moreover, the patient had immunological abnormalities and decreased neutrophilic functions (chemotaxis and O2- generation). Thus, it was suggested that the cutaneous lesions of this patient could be diagnosed as "neutrophilic dermatosis of MDS", and corticosteroid was recognized to be very effective in treating these skin lesions.

Anemia, Refractory↗

Crohn's colitis with extraintestinal manifestations: a case report.

The etiopathogenesis of inflammatory bowel disease remains unclear, with various extraintestinal features often found with the disease. Sometimes more than one extraintestinal finding is found in the same patient. In this case report, a patient with more than one extraintestinal manifestation of Crohn's disease is presented. Extensive thrombi in the venous system and pyoderma gangrenosum in the left lower extremity was observed but there was complete recovery following treatment with both systemic and topical agents.

Journal Article↗

Autoinflammatory syndromes.

The autoinflammatory disorders are a new and expanding classification of inflammatory diseases characterized by recurrent episodes of systemic inflammation in the absence of pathogens, autoantibodies or antigen specific T cells. These disorders are caused by primary dysfunction of the innate immune system, without evidence of adaptive immune dysregulation. Innate immune abnormalities include aberrant responses to pathogen associated molecular patterns (PAMPs) like lipopolysaccharide and peptidoglycan, prominent neutrophilia in blood and tissues, and dysregulation of inflammatory cytokines (IL-1beta, TNF-alpha) or their receptors. The autoinflammatory diseases comprise both hereditary (Familial Mediterranean Fever, FMF; Mevalonate Kinase Deficiency, MKD; TNF Receptor Associated Periodic Syndrome, TRAPS; Cryopyrin Associated Periodic Syndrome, CAPS; Blau syndrome; Pyogenic sterile Arthritis, Pyoderma gangrenosum and Acne syndrome, PAPA; Chronic Recurrent Multifocal Osteomyelitis, CRMO) and multifactorial (Crohn's and Behçet's diseases) disorders. Mutations responsible for FMF, TRAPS, CAPS, PAPA are in proteins involved in modulation of inflammation and apoptosis.

Autoimmune Diseases↗

Clinical characteristics of inflammatory bowel disease in Thailand: a 16 years review.

OBJECTIVE: Inflammatory bowel disease is an uncommon gastrointestinal problem in Asia. Recently, there have been many reports from Japan and India demonstrating a higher incidence of this disease entity in this region. To date, there has been only one report from Thailand regarding clinical manifestation of ulcerative colitis. However, information regarding Crohn 's disease has never been demonstrated. MATERIAL AND METHOD: The authors retrospectively reviewed clinical data on both ulcerative colitis (UC) and Crohn's disease (CD) during a 16 year period from the medical records of King Chulalongkorn Memorial Hospital, one of the main referral centers in Thailand. RESULTS: There were 55 patients diagnosed as inflammatory bowel disease. Of these, 45 were diagnosed as UC and the rest were CD. Mean age of UC and CD patients was 32 and 40.5 years respectively. The authors did not find any significant relationship between smoking and colonic cancer in these patients. The main presentations of UC patients were mucous bloody diarrhea and watery diarrhea whereas watery diarrhea, fever and weight loss were the main presentations of patients with CD. There were fistulas in 2 CD patients. In addition, oral ulcer and panuveitis were diagnosed in 2 different CD patients. One UC patient had PSC (primary sclerosing cholangitis) and another had pyoderma gangrenosum. The majority of UCpresented as pancolitis (46.7%) while ileocolic involvement was more common in CD (60%). CONCLUSION: In Thailand, UC is more common than CD whereas disease involvement is more localized to ileocolic region in CD than UC. Similar to Western countries, both UC and CD are presented at relatively young age.

Adult↗

[Association lichen planus and ulcerative colitis. A case report].

Ulcerative colitis is a frequent chronic inflammatory boxel disease. Its cutaneous manifestation are varied. Some of them are frequent like pyoderma gangrenosum, erethema nodosum and oral ulceration. We report the case of an exceptional association of ulcerative colitis with lichen planus in a 40-year old woman with a 2 year history of ulcerative colitis, who had lesions on the buccal mucosa and the lower lip for which the diagnosis of lichen planus was made and confirmed histologically.

Adult↗

Fusariosis occurring in an ulcerated cutaneous CD8+ T cell lymphoma tumor.

Fusarium species have recently emerged as the second most common pathogenic mold in immunocompromised patients, and they are moderately resistant to most antifungal agents. The skin lesions of disseminated fusariosis typically manifest as multiple red or violaceous macules or nodules, often ulcerated and covered by a black eschar. We report a case of cutaneous fusariosis in a patient with long-standing hypopigmented mycosis fungoides. The infection was successfully treated with a 3-month course of oral voriconazole. The present case is unusual in that the infection occurred within a pre-existing, ulcerated lesion of cutaneous CD8+ lymphoma, resulting clinically in confusion with pyoderma gangrenosum and necrosis of lymphoma. A high index of suspicion will prompt a timely biopsy as well as isolation of the fungus, and early institution of systemic antifungal therapy.

Antifungal Agents↗

[Chronic inflammatory bowel disease and cardiovascular complications].

The most common extraintestinal manifestations of Crohn's disease and ulcerative colitis are iritis and uveitis, primary sclerosing cholangitis (PSC) and nodal erythema and pyoderma gangrenosum. Complications within the cardiovascular system seem to be uncommon, but there are no systematic investigations concerning the epidemiology of these manifestations. There are more than 100 cases reported about pericarditis and perimyocarditis in patients with inflammatory bowel disease. Other patients with Crohn's disease or ulcerative colitis suffer from vasculitis, representing a further mechanism of inflammatory diseases of the cardiovascular system. There are several case reports showing a combination of Takayasu's arteritis and Crohn's disease, and cross-reacting antibodies against gut mucosa and aortic tissue were found. Some patients developed thrombotic complications by activating the coagulation system, which can result in atrial thrombi, embolism of the pulmonary arteries, myocardial infarction and disseminated intravascular coagulopathy (DIC). Furthermore, a few case were reported about atrio ventricular blocks, amyloidosis of the heart, dilative cardiomyopathy and endomyocardial fibrosis in patients with chronic inflammatory bowel disease. Here, a 27-year-old patient with known ulcerative colitis for 2 years is reported, who presented in the authors' department with unstable angina pectoris. Coronary angiographic examination was immediately performed and diffuse intracoronary thrombi were found, which could be removed by the catheter procedure. A myocardial infarction did not develop. Because of positive anti neutrophil cytoplasmic antibodies (p-ANCA) a p-ANCA-positive arteritis of the coronary vessels with intracoronary thromboembolism due to ulcerative colitis was diagnosed. Systematic studies or investigations concerning the epidemiology of the cardiovascular complications are still lacking, so that an overview about the published data is given.

Adult↗

Lupus anticoagulant and the skin. A longterm follow-up study of SLE patients with special reference to histopathological findings.

Skin manifestations were described in lupus anticoagulant (LA) positive and in LA negative SLE patients. Necrotic ulcers appearing at the beginning of the disease process characterized the 33 LA positive patients. Thirteen patients had a "peripheral vascular syndrome"; small leg ulcers of livedoid vasculitis type following deep venous thromboses, in 3 patients developing into pyoderma gangrenosum like ulcers and in 2 patients into pseudo-sarcoma Kaposi. The lesions were histologically characterized by capillary angiogenesis with extravasated red blood cells, sparse inflammatory cell infiltrates and microthromboses. Three patients had ulcers clinically and histologically resembling those seen in Degos' disease. Five patients had anetoderma showing elastic tissue depletion and microthromboses histologically. A different pattern of skin changes was seen in the LA negative patients. Our findings suggest that antiphospholipid antibodies play a pathogenetic role in the described skin manifestations of LA positive SLE patients.

Adolescent↗

Guidelines for treatment with infliximab for Crohn's disease.

Infliximab is an accepted induction and maintenance treatment for patients with Crohn's disease. The effectiveness of infliximab has been demonstrated for both active luminal disease and for enterocutaneous fistulisation. In addition, infliximab can be administered for extraintestinal symptoms of Crohn's disease, such as pyoderma gangrenosum, uveitis and arthropathy. Maintenance treatment with infliximab is effective and is regarded as safe as long as the necessary safety measures are heeded. Infusion reactions occur in 3 to 17% of the patients and are associated with the formation of antibodies to infliximab. A reduction in infusion reactions is possible by the concurrent administration of steroids and the use of immunosuppressants (azathioprine, 6-mercaptopurine, methotrexate). Furthermore, immunosuppressants increase the duration of the response to infliximab. For these reasons, the concomitant use of immunosuppressants with infliximab is recommended. Infections and most specifically tuberculosis need to be ruled out before infliximab is administered. Up to now, there are no indications for a connection between an increased risk for malignancies and treatment with infliximab.

Algorithms↗

Primary biliary cirrhosis associated pustular vasculitis.

The association between primary biliary cirrhosis (PBC) and cutaneous vasculitides is well recognized. Pustular skin lesions though, have been described in association with hepatobiliary diseases other than PBC. Once the more common infective pustular rashes have been excluded, the differential diagnoses for a pustular skin rash are acute generalized exanthematous pustulosis (AGEP), Sweet's Syndrome (SS), pyoderma gangrenosum (PG) and pustular vasculitis(PV). We present a case of pustular vasculitis associated with PBC.

Aged↗

Newer aspects of inflammatory bowel disease and its cutaneous manifestations: a selective review.

Idiopathic inflammatory bowel disease (IBD) denotes several types of gastrointestinal disorders characterized by protracted inflammation of the large or small bowel, and a tendency toward relapse or recurrence. The two major groups of IBD are identified as chronic nonspecific ulcerative colitis (UC) and Crohn's disease (CD). Extraintestinal manifestations of IBD are diverse and may antedate, occur with, or postdate the onset of the disease process itself. Cutaneous diseases such as erythema nodosum or pyoderma gangrenosum are not infrequent, and test the clinician's ability to recognize the underlying bowel pathology and institute appropriate treatment. This selective review will address the immunological pathogenesis of IBD, new or rarely reported skin markers in IBD, drug therapy and side effects, zinc deficiency, management of osteotomy patients, and cutaneous cancer complicating IBD. Although the prognosis of IBD has improved, the role of the dermatologist as a consultant remains invaluable in reducing the attendant cutaneous morbidity.

Humans↗

A review and update of the clinical uses of cyclosporine in dermatology.

Cyclosporine is a hitherto unique immunosuppressive agent that has provided a new approach to therapy in autoimmune diseases and has been increasingly used as a molecular tool for the investigation of immune regulation. It has produced excellent therapeutic responses in the treatment of psoriasis, acrodermatitis continua of Hallopeau, pyoderma gangrenosum, lichen planus, pityriasis lichenoides chronica, Behçets disease, and alopecia areata. Cyclosporine's mechanism of action, side effects, pharmacokinetics, and guidelines for patient monitoring, are discussed in this article.

Alopecia↗

[Sternal osteomyelitis associated with a severe attack of ulcerative colitis with multiple extra-intestinal manifestations].

A case of ulcerative colitis in which multiple extraintestinal manifestations: erythema nodosum, pyoderma gangrenosum, vesicle pustule lesions, aseptic osteomyelitis and possible local myositis appeared in the course of an acute decompensation is presented. The pathogenesis of these lesions are discussed and the not previously reported association between ulcerative colitis and aseptic osteomyelitis is presented.

Colitis, Ulcerative↗

[Vesiculopustular eruption and Sweet syndrome associated with 2 exacerbations of ulcerous colitis in a 76-year-old woman].

The concept of acute febrile neutrophilic dermatosis (Sweet's syndrome) has evolved and expanded to include, under the denomination of neutrophilic dermatosis (ND), both pyoderma gangrenosum and the intestinal bypass syndrome, with or without intestinal bypass, vesicular and pustular rash associated with ulcerative colitis (UC), and Sweet's syndrome itself. Among the skin lesions associated with UC several types of vesicular and pustular rash have been reported and, very rarely, the association of UC and Sweet's syndrome has been reported. We report a female with UC who developed a vesicular and pustular rash associated with an exacerbation of her colonic disease. Two years later she developed a characteristic Sweet's syndrome coincident with another exacerbation of UC. The concurrence of two different forms of ND associated with successive exacerbations of UC supports the concept of ND as an individual condition with several types of clinical presentation.

Aged↗