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[Thalidomide: an eclectic medication in dermatology].

The authors review the indications for the use of thalidomide in dermatological conditions. They consider leprotic reaction, prurigo nodularis of Hyde, actinic prurigo, discoid lupus erythematosus, Weber-Christian panniculitis, recurrent mouth aphtosis, Behcet's syndrome, and pyoderma gangrenosum. The dramatic situation created by the use of thalidomide in the sixties not-withstanding, interest in its use is warranted within certain limits due to its wide therapeutic action.

Humans↗

[Ischemic and inflammatory colitis in elderly subjects over 70].

Twenty-five cases of non-infectious, macroscopic colitis, observed consecutively in patients over 70, were studied. The final diagnosis was, in 12 instances, ischemic colitis, in 10 instances cryptogenetic colitis (6 ulcerative colitis and 4 Crohn's diseases), and in 3 instances, non-classified colitis. The clinical manifestations consisted of rectal bleeding (92%), abdominal pain (76%) and diarrhea (56%). In 3 cases, the ano-perineal lesions observed in Crohn's diseases were noted and, in one case, a pyoderma gangrenosum was present. Endoscopy and/or barium enema showed a lesion of the left colon in 92% of the cases and of the sigmoid colon in 80% of the cases. The prognosis was severe, requiring surgery in 32% of the cases (5 ischemic colitis, 3 cryptogenetic colitis), and a 24% mortality (3 ischemic colitis, 3 ulcerative colitis), secondary to the condition and the extent of the colic involvement. The best factors of the etiological diagnosis of colitis were: the type of onset of the symptoms, the presence of anal or cutaneous lesions, the information provided by endoscopy and histology, which were specific in 11 out of 20 cases. In 5 cases, the diagnosis was only made by examination of the surgical specimen; in 3 cases, the diagnosis remained uncertain. These results suggest that the clinical data combined with an early, minimally traumatizing left endoscopy in elderly patients, should allow a quick, positive and etiological diagnosis of colitis, in order to initiate a suitable treatment in most cases.

Aged↗

[Acute febrile neutrophilic dermatosis and malignant hematologic diseases: report of a new bullous case and review of the literature].

A new case of Sweet's syndrome (acute febrile neutrophilic dermatosis) associated with a malignant hemopathy is presented. The blood disease was a chronic myelomonocytic dysmyelopoiesis which was discovered during the eruption and resulted in the patient's death within a few months, probably through acutization. The skin lesions were atypical, bullous and ulcerated. On this occasion, the international literature concerning all cases of Sweet's syndrome associated with malignant or premalignant hemopathies is reviewed. Several concepts emerge from this study: the association is frequent (about 20 p. 100 of all published cases of Sweet's syndrome); there is a strong predominance of granulocytic hemopathies over lymphoplasmocytic and monocytic hemopathies; the blood disease is revealed by the skin eruption in some 50 p. 100 of the patients; there are frequent chronological relations between Sweet's syndrome and the events that occur in the course of the hemopathy; finally, the association is usually of poor prognosis. A comparison with Sweet's syndrome unassociated with a blood disease showed only three significant points: the frequency of bullous lesions, of the initial anaemia (the most important element) and of extreme figures in leucocyte counts (leucopenia or major hyperleukocytosis). The atypical character of the skin lesions in the patient presented here incites to discuss the nosological relationship between Sweet's syndrome and bullous pyoderma, an entity closely associated with hemopathies. It has recently been suggested by several authors that this anatomico-clinical kinship should be turned into a wide spectrum of acute neutrophilic dermatoses, with typical Sweet's syndrome at one end and Pyoderma gangrenosum at the other end. The interface between this spectrum and haemopathies seems to be maximum at its intermediate stage: the bullous and superficially ulcerated lesions. The aetiology and pathogenesis of this new nosological entity are uncertain. The presence of chemoattractants or of polymorphonuclear cell abnormalities is still open to discussion. The relationship between the entity and leukocytoclastic vasculitis has recently been questioned.

Aged↗

[Cutaneous manifestations of ulcerative colitis].

Out of 210 cases of ulcerative colitis 41 had skin and mucosal lesions: skin lesions in 23 (11%), oral ones in 11 (5.2%) and perianal in 16 (7.6%). Cutaneous lesions were: maculopapular rashes (5.2%), erythema nodosum (2.9%), pyoderma gangrenosum (1.4%), papulopustular lesions, vasculitis and erythema multiforme (1% each). Aphthae were the commonest oral complication (4.3%) and fissures and fistula the more prevalent perianal ones (4.3% and 3.4%). More than one mucocutaneous complication occurred in 17.1% of the patients and in 18 patients there were associated articular (36.6%), ocular (9.8%) and hepatic (2.4%) changes. The colitic type of arthritis and keratoconjunctivitis sicca were the commoner joint and eye manifestations.

Colitis, Ulcerative↗

Atypical neutrophilic dermatosis with subcorneal IgA deposits.

A 26-year-old woman had a chronic vesiculopustular and ulcerating skin disease associated with fever and arthritis. Cutaneous biopsy specimens showed an extensive infiltration of the dermis and epidermis by neutrophils. Direct immunofluorescence (IF) revealed linear subcorneal IgA deposits. Indirect IF showed IgA antibodies reactive with the subcorneal zone of normal epidermis. The disease responded to dapsone therapy. The association between neutrophilic dermatoses, including pyoderma gangrenosum, subcorneal pustular dermatosis, and related entities, and IgA involvement, either IgA gammopathies and/or intraepidermal IgA deposits, is emphasized. Intraepidermal IgA deposits are possibly involved in the pathogenesis of our patient's condition and of other cases of unusual neutrophilic dermatoses.

Adult↗

Transient subcutaneous granulomatosis of the upper extremities in Crohn's disease.

Skin lesions occur fairly commonly in patients with Crohn's disease of the colon (14%). Erythema nodosum and pyoderma gangrenosum are the most frequently recognized. Granulomatosis of the skin, continuous with bowel involvement, has been reported. Involvement of the subcutaneous tissue, discontinuous with intestinal disease, is unusual and we wish to report a case of Crohn's disease of the colon complicated by infraepidermal perivascular granulomas of the forearms. The lesions disappeared spontaneously.

Adult↗

Bromoderma caused by carbromalhydroxyzine hydrochloride.

A patient is described who developed a pyoderma-gangrenosum-like ulcer of the leg and acneiform eruption on the face following intake of carbromalhydroxyzine hydrochloride (Dormax). Serum levels of bromide were found to have risen to 105 mg%. There was a return to normal levels of serum bromide and regression of the skin lesions after one week of treatment with natrium chloride and erythromycin. The importance of increasing awareness of the potential hazards of bromide administration is stressed.

Adult↗

[Should thalidomide be rehabilitated?].

Thalidomide (alpha-N-phtalimido-glutarimide) was withdrawn from sale in 1961 since it was held responsible for the birth of hundreds of phocomelus children. Despite this teratogenic potential, thalidomide remains a useful tool in the treatment of erythema nodosum leprosum, as well as in discoid lupus erythematosus when antimalarial drugs are ineffective or contraindicated. In addition, good results have been reported in several diseases such as actinic prurigo, polymorphous light eruption, Behçet syndrome, Weber-Christian disease, prurigo nodularis, pyoderma gangrenosum and ulcerative colitis. The mechanism of action is under debate but it is likely that thalidomide has immunomodulating properties by controlling T-suppressor lymphocytes, and anti-inflammatory effects, particularly an inhibition of neutrophil chemotaxis. Several attempts at synthesis of effective thalidomide derivatives devoid of teratogenic effects are ongoing.

Chemical Phenomena↗

Metastatic cutaneous Crohn's disease.

A 16-yr-old boy and a 38-yr-old woman with Crohn's disease developed granulomatous dermatitis on the forearm and lower calf, respectively. Their lesions were clinically and histologically similar to those described in the small number of prior reports of "metastatic" cutaneous Crohn's disease. They were readily distinguished from those of other dermatologic conditions associated with Crohn's disease, such as pyoderma gangrenosum, erythema nodosum, and cutaneous polyarteritis nodosa. Appropriate clinical studies, biopsy specimens, and cultures are required, however, to exclude other forms of granulomatous disease that could occur coincidentally in a patient with Crohn's disease.

Adolescent↗

[Skin changes in paraproteinemias (monoclonal gammopathies)].

Numerous skin disorders or skin lesions are associated with a monoclonal gammopathy (formerly: paraproteinemia). These skin diseases can be differentiated in the following way: specific skin lesions caused by monoclonal immunoglobulin producing cells in the skin, in generalized plasmocytoma or morbus Waldenström. In another group of disorders the monoclonal immunoglobulins induce functional disturbances, e.g. Raynaud syndrome (if the monoclonal immunoglobulins are cryoglobulins), xanthomatosis through interactions between these immunoglobulins and lipoproteins, purpura in immunoglobulin induced stasis and hyperviscosity. The unlimited spreading of the plasmocytoma cells replaces normal immunoglobulin producing plasma cells followed by a disturbance of the humoral immune response, which is frequently accompanied by atypical infections of skin or other organs. Furthermore, an almost obligatory association with monoclonal gammopathies has been demonstrated in a few skin diseases, e.g. scleromyxedema. However, the role of the immunoglobulins in these disorders is unknown. In some other skin diseases, such as pyoderma gangrenosum, acrodermatitis atrophicans Herxheimer, scleredema Buschke etc., a monoclonal gammopathy is only detectable in some patients.

Amyloidosis↗

[Huge gangrenous cellulitis. Clinical symptoms-differential diagnosis--therapy].

Extensive necrotizing cellulitis occurs in adults, when body resistance is reduced by serious general disorders. Differential diagnosis should exclude the following diseases: gangrenous erysipelas, necrotizing fasciitis, gangrenous ecthyma, pyoderma gangrenosum, purpura fulminans and Coumarin necrosis. In addition to antibiotic therapy, the removal of vast necrotic areas as well as plastic covering is necessary. A case of typical predisposing disorders, clinical symptoms and development of the disease is reported.

Aged↗

Vasculitis involving muscle associated with Crohn's colitis.

A case of Crohn's colitis is described in a patient who demonstrated multiple extraintestinal complications, including erythema nodosum, pyoderma gangrenosum, and aphthous oral ulcerations. Our patient simultaneously developed an unusual vasculitis in muscle. Small vessels were affected by necrosis, and polymorphonuclear leukocyte infiltration was appreciated. Leukocytoclastic was suggested by these features and the additional finding of small amounts of nuclear debris. A strong immunofluorescence to antihuman C3 was demonstrated in the affected vessel walls. We did not find circulating IgG immune complexes in serum with the Raji cell assay. The implication of the finding of leukocytoclastic vasculitis in muscle, associated with Crohn's colitis, is discussed.

Adult↗

Avulsion of the quadriceps tendons in a patient with an unusual deforming arthritis and varied skin lesions.

Spontaneous avulsion of both quadriceps tendons occurred in a young woman who had been treated with corticosteroids for progressive severely deforming seronegative arthritis and recurring skin lesions clinically and histologically suggestive of cutaneous vasculitis and pyoderma gangrenosum. We postulate that recurrent minor trauma to tendons previously damaged by vasculitis and inhibition of tendon repair by oral corticosteroids were predisposing factors to the bilateral tendon avulsions.

Adrenal Cortex Hormones↗

Treatment of leg ulcers with cultured epithelial autografts: clinical study and case reports.

Leg ulcers are a significant medical problem in the United States. The number of affected patients at any given time is staggering. Treatment modalities have widely varied and are often unsuccessful. We evaluated the effectiveness of cultured epithelial autografts in the treatment of leg ulcers due to various etiologies that had failed other standard therapy. Eighty-six ulcers in 36 patients were treated with cultured epithelial autografts after full evaluation of their ulcers. The ulcers were grouped by etiology and healing was assessed by time to healing, number of ulcers healed and mean number of graft applications. The results achieved in the past five years have shown that cultured epithelial autografts are highly effective in treating chronic leg ulcers. Eighty percent of venous insufficiency ulcers healed in 5.7 weeks. Similar results were seen with ulcers of other etiologies including arterial insufficiency, pyoderma gangrenosum, scleroderma, and vasculitis. Positive results were seen in cases where other treatment methods had failed and seems independent of systemic corticosteroids.

Adult↗

[Skin symptoms in gastrointestinal diseases].

Diseases of the skin and the gastrointestinal tract may occur together. It is important to examine the skin of everyone showing a gastrointestinal problem. Gastrointestinal signs and symptoms in dermatologic diseases may occur with dysphagia, abdominal pain, gastrointestinal bleeding and diarrhea with or without malabsorption. In general the cause is found in a genetic disorder, or it is infectious, drug-induced, inflammatory or related to a malignant disorder. Polyposis are hamartomatous tumors or result as an inflammatory reaction. All these syndromes may present with cutaneous lesions. As malignant degeneration of polyps often develops, the early diagnosis and preventive treatment is crucial. Inflammatory bowel disease is often associated with skin complications such as pyoderma gangrenosum and erythema nodosum. Malignant disorders in the gut may metastasize into the skin or may produce rather typical paraneoplastic changes.

Colitis↗

[Skin lesions after injection abuse. Chronic changes caused by ketobemidone (Ketogan)].

Local complications of repeated injections of pentazocine are known among drug abusers. We hereby describe similar reactions in three patients who for several years had abused the opioid analgetic ketobemidone for chronic pain after abdominal surgery, and in six drug addicts who regularly injected ketobemidone tablets dissolved in water. In all nine patients an impressive clinical picture with disfiguring woody fibrosis and multiple irregular ulcers and scars surrounded by hyperpigmentation on the upper and lower extremities was seen. The skin lesions had been mistaken for aggressive forms of panniculitis, pyoderma gangrenosum or selfmutilation. Following direct questioning most patients admitted self-administration of the drug to the affected areas. The patients showed an apparent indifference about their extensive disabling skin disease, and no obvious complaints of pain, contrary to what might be expected from the pronounced tissue destruction and inflammation at the injection sites.

Aged↗

Cutaneous paraneoplastic syndromes.

Cutaneous paraneoplastic syndromes are a large group of dermatoses that may be associated with an internal malignancy. Among these dermatoses are acanthosis nigricans, tripe palms, dermatitis herpetiformis, dermatomyositis, extramammary Paget's disease, hypertrophic pulmonary osteoarthropathy, pemphigus vulgaris, pruritus, pyoderma gangrenosum, Sweet's syndrome and reactive erythemas. Once the diagnosis of the dermatosis has been confirmed, an appropriate work-up should be undertaken to search for an underlying asymptomatic neoplasm in a patient without known cancer or to detect recurrent or metastatic disease in a patient with an established history of malignancy.

Humans↗

[Diagnostic pitfalls of cutaneous complications of chronic venous insufficiency].

Cutaneous lesions of the lower limbs are not always of venous origin. Only careful examination of the skin and of the leg can detect other causes. Such examination is important not only for understanding the pathophysiological origin but especially for treatment decision. Certain possible causes should be routinely sought such as arterial abnormalities and cutaneous vasculitis. Some ulcerations from the onset have an atypical appearance, such as necrotic angiodermatitis and pyoderma gangrenosum. Rarer causes (tumoural, haematologic, infectious or congenital) should be considered in case of resistance to usual treatments for severe venous insufficiency.

Chronic Disease↗