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At least 1,351 records · Page 75Linked to original sources
PubMed1993

Immunosuppressive agents in dermatology.

Azathioprine, cyclophosphamide, and cyclosporine are immunosuppressive agents commonly used by dermatologists. Azathioprine has a good safety profile and is preferred by most dermatologists for the treatment of bullous pemphigoid, cutaneous vasculitides, chronic eczematous dermatitides, and cutaneous manifestations of connective tissue diseases. For more fulminant diseases such as Wegener's granulomatosis, lymphomatoid granulomatosis, severe cases of systemic lupus erythematosus, or pemphigus, cyclophosphamide is more effective and its use is justified even though it is more toxic. Cyclosporine has been shown to be effective in a variety of dermatoses; the most promising results are seen in psoriasis, lichen planus, pyoderma gangrenosum, and chronic eczematous dermatitides. The long-term safety of cyclosporine remains to be determined, and it should be used judiciously with careful evaluation of the risk-benefit ratio in each case. Dermatologists who use immunosuppressive agents should be familiar with their indications, side effects, dosage, and monitoring.

Azathioprine↗
PubMed1993

[Crohn disease: morphologic findings of extra-intestinal disease manifestations].

Extraintestinal manifestations occur frequently in patients with Crohn's disease. The spectrum of extraintestinal symptoms reported associated with Crohn's disease involves many organ systems. Commonly recognized extraintestinal manifestations include dermatologic, oral, ocular, skeletal, vascular, hepatobiliary, pancreatic, and pulmonary. Morphological findings on extraintestinal manifestations (erythema nodosum, pyoderma gangrenosum, erythrodermia--granulomatous periostitis and synovitis--granulomatous sialadenitis, aphthous stomatitis--fibrous alveolitis) in Crohn's disease are reported and discussed.

Biopsy↗
PubMed1995

Extraintestinal considerations in inflammatory bowel disease.

If one reviews the literature with zeal, it is increasingly apparent that few organs escape recruitment when IBD is chronic or progressive. Insights into mucosal pathophysiology have helped with understanding the more frequent extraintestinal manifestations, but the mechanisms attendant to the development of less common events (e.g. acute pancreatitis, concurrent gluten sensitive enteropathy, or active pulmonary disease) remain either poorly studied or obscure. It is particularly interesting, however, to read reports of abnormal pulmonary function, generally of the obstructive type, correlated to measurements of abnormal intestinal permeability in patients with either active pulmonary sarcoid or pulmonary involvement in Crohn's disease. It has been further speculated that similarities in the mucosal immune system of the lung and intestine are responsible for evidence of bronchial hyperreactivity in patients with active IBD. Finally, it is important to recognize that extensions of the inflammatory process are not restricted to the development of organ-based events but may be responsible for some of the most frequent systemic abnormalities detected in IBD patients. It is now also well confirmed that the cytokine environment in IBD can support activated coagulation and, in some clinical situations, overt vascular thrombosis. The cerebrovascular complications of IBD are well recognized and range from peripheral venous thrombosis to central stroke syndromes and pseudotumor cerebri. Reports of focal white matter lesions in the brains of patients with IBD or an increased incidence of polyneuropathy may be other clinical examples of regional microvascular clotting. Microvascular injury appears to be more ubiquitously present, with reports ranging from a speculated primary causative role (e.g., granulomatous vasculitis in the mesenteric circulation) to the utility of nailbed vasospasm, in Crohn's disease, as a clinical marker for disease activity. It is also reported that IL-6 suppression of erythropoietin production is a major feature of the chronic anemia seen in active IBD. Moreover, the capacity of peripheral monocytes from active IBD patients to secrete TNF and IL-8 is reported predictive for the degree of therapeutic response from recombinant erythropoietin. These collected observations constitute another excellent example of the symmetry between basic science and clinical utility. It is from the context of applied basic science that many future therapies will arise. Empiricism will lose much of its appeal as clinical observations will be increasingly translated into cellular language. Already in animal models, elemental diets diminish IL-6-related acute inflammatory injury, and reductions in dietary lipid alter the antigenicity of bacteria. Provocatively, in humans, unconfirmed reports have even associated diet therapy with the resolution of uveitis and pyoderma gangrenosum. It is likely that efforts will also be made to induce oral tolerance if specific triggering proteins are discovered or to alter bowel flora if such an arcane area of investigation becomes resurgent.

Anemia↗
PubMed1997

[Sweet's syndrome: a study of 16 cases].

We performed a retrospective analysis of the records of 16 patients diagnosed in our dermatology service as Sweet's syndrome (SS), with the aim of describing their clinical findings and associations, and comparing our results with previous ones. The mean age was 51, and 82% were female. A previous infection was recorded in 5 cases (31%) (gastroenteritis, primary pulmonary tuberculosis, upper airways infection, wound infection, and streptococcal pharyngitis). Two patients (12%) suffered from a malignant neoplasia (acute myeloid leukemia and prostatic neoplasia), another patient had a coincident bout of acute ulcerative colitis with pyoderma gangrenosum, and a third one referred previous ingestion of diclofenac and intense sun exposure. Most patients had their lesions localized on the upper extremities (75%), fever was present in 8 cases (50%), arthralgia in six (37%), and erythema nodosum in five (31%). The most frequent laboratory finding was an elevated sedimentation rate (93% had values over 20 mm/h), and only 44% of patients had leukocyte counts over 10 x 10(9)/l. Urinanalysis was abnormal in one third of the patients, and chest roentgenograms, performed in ten patients, were all normal. Most of the patients were treated with low doses of oral prednisone (30 mg/day) with good results. The disease recurred in 25% of the patients.

Adult↗
PubMed1998

Cutaneous sporotrichosis presenting as a keratoacanthoma.

Sporothrix schenckii is a ubiquitous dimorphic fungus with a variety of clinical presentations, including imitation of other diseases such as pyoderma, rosacea, and pyoderma gangrenosum. A case in which sporotrichosis appeared as a keratoacanthoma-like nodule on the lower lip of a young woman is presented, with treatment considerations and diagnostic pitfalls.

Adult↗
PubMed1997

[Inflammatory intestinal diseases and their extra-intestinal manifestations observed at Liban].

101 cases classified as inflammatory bowel disease at the Hôtel-Dieu de France Hospital between 1982 and 1994 were investigated. Files containing a sure diagnosis and complete clinical and biological investigations were included. 65 files were retained for the study: 48 cases had ulcerative colitis (UC), 17 had Crohn's disease (CD). UC averaged 4.7 admissions per 10,000 admissions to the hospital, while CD averaged 1.54 per 10,000. The UC/CD ratio was 2.8 and the Female/Male ratio was 1.4 for UC and 0.9 for CD. Medium follow-up was 6.4 years. 30% of our group was followed for more than 10 years. Overall frequency of extra-intestinal manifestations was similar to that reported worldwide. This is especially true for UC. However, no cases of ankylosing spondylitis, sclerosing cholangitis or erythema nodosum were found. One case of pyoderma gangrenosum was found in the series of CD. Extra-intestinal manifestations were found in 54% of UC patients and 94% of CD patients, probably because milder cases of this disease were misclassified as infectious diarrhea.

Adult↗
PubMed1998

[Differential ulcus cruris diagnosis].

Most leg ulcers are of venous or arterial origin (85%). Advanced chronic venous insufficiency is the most common underlying condition (65%), followed by advanced peripheral arterial occlusive disease (10%), and combined chronic venous insufficiency and peripheral arterial occlusive disease (10%). Chronic ulcers in diabetic feet (5%) are of great socio-economic importance, as well. They are a consequence of diabetic polyneuropathy which in part of the patients may be combined with peripheral arterial occlusive disease, usually of the calf arteries. However, a leg ulcer can also be caused by a large array of other underlying conditions, such as ulcerating skin tumours, trauma followed by disturbed wound healing, infectious ulcerations, ulcerations in angiodysplasias, vasculitic ulcerations, pyoderma gangrenosum, cholesterol-embolism, idiopathic livedo reticularis with ulceration, primary and secondary antiphospholipid-antibody-syndrome, coumarin-necrosis, calciphylaxis in chronic renal insufficiency, necrobiosis lipoidica, different forms of panniculitis, hematologic disorders, autoimmun diseases and autoimmun-bullous dermatoses. The following article discusses the differential diagnosis, examination and treatment of leg ulcers in these less common underlying conditions.

Diagnosis, Differential↗
PubMed1998

[Purpura--an unusual presentation of Takayasu arteritis].

Takayasu arteritis is an uncommon vasculitis, often referred to as aortic arch syndrome. It is most prevalent in young women. Physical findings such as the combination of pulseless arms and carotid bruits suggest the diagnosis. Associated skin manifestations such as pyoderma gangrenosum and erythema nodosum have been described. We present a 27-year-old woman with diffuse purpuric eruption and Takayasu arteritis. This appears to be the first description of such an association.

Adult↗
PubMed1990

[Livedo with cerebrovascular thrombosis--correlation between clinical features, anti-cardiolopin antibodies, and cerebral microinfarction].

Twenty one patients with livedo racemosa (LRa) and periarteritis nodosa cutanea were screened for the presence of anti-cardiolipin antibody (aCL) by ELISA. 11 out of 21 revealed positive aCL whose clinical features could be divided into 3 groups: 4 patients with livedo with ulceration, 2 with pyoderma gangrenosum-like lesions, and 5 with extensive LRa. #1. The summer ulceration of leg of 4 patients were surrounded by several tiny petechiae where hyaline microthrombi of blood capillaries in the upper dermis were demonstrated. All had multiple cerebral microinfarctions on magnetic resonance imaging (MRI). #2. Pyoderma gangrenosum-like lesions of 2 patients consisted of painful punched-out ulcers on livedoid lesions where intravascular endothelial hyperplasia in a small artery was demonstrated. Both had multiple cerebral microinfarctions. #3. Another clinical manifestation of the patients with positive aCL was extensive form of LRa of 5 patients. All had positive ANA, however none of them exhibited cerebral microinfarctions on MRI. #4. The remaining 10 patients with negative aCL showed no relationship with such clinical and laboratory manifestations. These results indicate that there is a significant correlations between summer ulceration with petechiae, capillary microthrombi of the skin, positive aCL and cerebral microinfarctions on MRI.

Adult↗
PubMed1996

Dermatological presentation of disease associated with antineutrophil cytoplasmic antibodies: a report of two contrasting cases and a review of the literature.

Antineutrophil cytoplasmic antibody (ANCA)-associated disease (AAD) constitutes a pathological disease spectrum of a necrotizing vasculitis of small- and medium-sized vessels, extravascular granuloma formation, and necrotizing and crescentic glomerulonephritis, and also a clinical disease continuum which ranges from renal-limited disease to a widespread systemic vasculitis, including Wegener's granulomatosis and microscopic polyangiitis. In the latter, circulating ANCA are an aid to diagnosis and also may play a pathogenic part. Two contrasting patients with AAD are described, both of whom presented primary with dermatological features. These included a cutaneous purpuric vasculitis, orogenital ulceration, infarction of the fingertip, and pyoderma gangrenosum-like ulceration. These cases will familiarize dermatologists with both the concept and dermatological features of AAD.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1997

[Microscopic polyangiitis with eosinophilia--an overlap syndrome or separate disease entity? A case report and review of the literature].

Systemic vasculitides are potentially life-threatening diseases. Early and appropriate diagnosis based on case history, clinico-pathological features, and laboratory parameters, such as the presence of anti-neutrophil cytoplasmic antibodies (ANCA), is crucial for starting appropriate and, often, life-saving therapeutic measures. We report a 50-year-old female patient who presented with fever, arthralgias and hemoptysis. Skin signs included disseminated hemorrhagic pustules, ulcerations of oral and genital mucosa, subcutaneous nodules on arms and legs, and a pyoderma gangrenosum-like lesion on the right leg. Laboratory investigations revealed a peripheral eosinophilia and a positive cANCA titer. Histopathologic analysis of various biopsy specimens showed a granulomatous vasculitis in the subcutis, a nongranulomatous vasculitis with massive eosinophil infiltration in the lungs, and a segmental, necrotizing glomerulonephritis in the kidneys. Differential diagnosis included Wegener's granulomatosis, microscopic polyangiitis (MPA) and Churg-Strauss syndrome. MPA was diagnosed based on clinical and histopathological criteria. An interesting feature of this case was marked peripheral and tissue eosinophilia. Therapy consisted of cyclophosphamide and methylprednisolone. The patient went into a long-lasting clinical remission one month after starting therapy.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1992

[Gangrenous ecthyma of the diaper area in infants].

Ecthyma gangrenosum due to Pseudomonas aeruginosa is a skin infection in which necrotic ulcerations surrounded by a red areola develop. The diaper area is the region most often involved in infants. Typically, ecthyma gangrenosum occurs in patients with septicemia and risk factors (chemotherapy, neutropenia). However, transient bacteremia or an infection confined to the skin may be the cause in some patients, with maceration in the diaper area and previous antibiotic therapy as risk factors.

Diagnosis, Differential↗
PubMed2002

[ANCA-positive vasculitis of the skin and kidneys associated with acne conglobata].

A 19 year old man with severe acne conglobata and ulcerated pyoderma gangraenosum-like skin lesions on the face was first treated with isotretinoin (Roaccutan((R))), then immunosuppressively with prednisolone, diaminodiphenylsulfone (Dapson-Fatol((R))) and mycophenolate mofetil (Cellcept((R))). Under a daily maintenance dose of immunosuppressive treatment with 2.5 mg prednisolone and 1 g mycophenolate mofetil, weakness, muscle and joint aches appeared. Due to proteinuria, haematuria and an elevated antineutrophil cytoplasmic antibody (cANCA) and the histological detection of a leukocytoclastic vasculitis, the diagnosis of cANCA positive vasculitis of the skin and kidneys was established. Therapy with cyclophosphamide (Endoxan((R))) along with prednisolone was effective. An exact classification of this disease process was not possible.

Acne Vulgaris↗