Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Intravenous immunoglobulins (IVIgs) exert a variety of immunomodulating activities and are, therefore, increasingly being used for the treatment of immune-mediated as well as autoimmune diseases. There is also accumulating evidence that high-dose IVIg (hdIVIg) is highly efficacious in the treatment of skin diseases, despite the lack of evidence from randomized, double-blind, placebo-controlled trials. A major advantage of hdIVIg in comparison with other commonly used immunomodulating therapeutic strategies is the excellent safety profile. Accordingly, IVIgs have been used successfully for the treatment of bullous autoimmune diseases such as pemphigus and bullous pemphigoid, dermatomyositis, scleroderma, cutaneous lupus erythematosus, toxic epidermal necrolysis, and erythema exudativum multiforme. In most cases, hdIVIg is effective only in combination with other immunomodulating strategies and allows for the reduction of adjuvants. Adverse effects of hdIVIg are generally mild and self-limiting. These include headache, myalgia, flush, fever, nausea or vomiting, chills, lower backache, changes in blood pressure, and tachycardia. To avoid infusion-related rigors, headaches, and other adverse events, pre-treatment with analgesics, NSAIDs, antihistamines, or low-dose intravenous corticosteroids may be beneficial. Controlled, double-blind, long-term clinical trials and a better understanding of the complex immunomodulating mechanism of IVIg are required to ultimately optimize dose, frequency, duration, and mode of IVIg administration.
 ...
PMID:Efficacy and safety of intravenous immunoglobulin for immune-mediated skin disease: current view. 1518 94

Blistering eruptions are rare cutaneous manifestations of lupus erythematosus (LE) that may be caused by different mechanisms. Subepidermal clefting with frank vesiculation may occur in early lesions of chronic-, subacute-, and acute-cutaneous LE due to a severe vacuolar alteration of the dermoepidermal junction (DEJ), dermal edema, and lekocytoclastic vasculitis. An exaggerated example of such changes is rarely seen at the advancing edge of the annular plaques of subacute cutaneous LE with erythema-multiforme (EM)-like appearance, a condition formerly described as Rowell's syndrome. In a recently reported novel variant of LE-associated toxic epidermal necrolysis, dysregulated keratinocyte apoptosis has been proposed as an underlying mechanism. These vesiculobullous lesions are considered to be LE-specific. Blistering may also occur in LE in the context of a coexisting immunobullous disease. Pemphigus, bullous pemphigoid (BP), epidermolysis bullosa acquisita (EBA), dermatitis herpetiformis, and linear IgA bullous dermatosis have been all reported in association with LE. Their differentiation relies upon characteristic clinical, histologic, and immunopathologic features (Table 1). These blistering eruptions are rather non-specific for LE.
 ...
PMID:Bullous systemic lupus erythematosus. 1523 14

Among the numerous idiopathic immune-mediated diseases that can be drug-induced, such as pemphigus, psoriasis, lichen, etc, drug-induced lupus is the most widely commented upon and investigated. The terms drug-induced lupus (DIL) and drug-induced lupus erythematosus (DILE) are preferred, but other ones are also used--drug-related lupus, lupus-like syndrome, and lupus erythematosus medicamentosus. This review discusses the general issues in DILE, such as pathogenic mechanisms, clinical forms, and diagnostic criteria, and provides more detailed information for some of the implicated drugs: minocycline, statins, terbinafine, etc.
 ...
PMID:Drug-induced lupus erythematosus. 1523 17

Few cases have been published relating systemic lupus erythematosus (SLE) and pemphigus vulgaris (PV). We describe a patient with this association. A 35-year old woman who started to develop persistent pain and morning stiffness of proximal inter and metacarpo-phalangeal joints. During the following year, the patient recalled the onset of blisters on both legs, face, arms and thorax, as well as erosions appearing on oral mucous membranes. We observed generalized multiple erosions on her trunk and legs, flaccid bullae located on her right thigh and multiple erosions on oral mucous membranes. A skin biopsy reported PV Direct immunofluorescence on the perilesional skin specimen, showed beehive intercellular IgG deposits in the epidermis (+++), suggesting PV; granular discontinuous IgM and C3 deposits in the dermal-epidermal union (+++), suggesting SLE. Direct immunofluorescence of the healthy unexposed skin specimen, reported granular discontinuous IgG deposits in the dermal-epidermal union and beehive intercellular IgG deposits in the lower levels of the epidermis (+++); granular continuous IgM deposits in the dermal-epidermal union (+++). The results of rheumatic studies were obtained as follows: ANA :3 +, Anti-DNA, Anti-Sm, Anti-Ro and Anti-La :4 + . The definite diagnosis was PVand SLE. Treatment with 50 mg of prednisone daily with good evolution.
Lupus 2004
PMID:Systemic lupus erythematosus and pemphigus vulgaris: association or coincidence. 1564 51

Case 1: A 33-year-old man with a 14-year history of localized skin disease on the face and scalp was evaluated at the department of dermatology. The physical examination revealed plaques with papules, pustules, and a golden yellow crusting on the forehead, cheeks, upper lip, and chin (Figure 1). The scalp presented fine, whitish scales. At the beginning of his disease, the patient presented large red and painful purulent boils. The 14-year clinical course of these lesions was characterized by partial remissions and recurrences, but he did not specify any treatment related to improvement. The clinical diagnosis given for the scalp lesions was seborrheic dermatitis. For the facial lesions, many differential diagnoses were considered, among them: seborrheic dermatitis, acneiform dermatitis, impetigo, folliculitis, seborrheic pemphigus, and demodicidosis. The histopathologic study of a biopsy taken from the cheek (Figure 2) showed superficial spongiform dermatitis with neutrophils and folliculitis that are compatible with the diagnosis of seborrheic dermatitis. Both Gram and periodic acid-Schiff stains were negative. Follow-up of the patient was not possible since he did not come back. The disease in this patient initially manifested at age five by the presence of recurrent ganglionic abscesses. At age 15, he presented a pulmonary abscess of a left lobule that was surgically removed; at this point the diagnosis of chronic granulomatous disease was established. At age 28, an exploratory laparotomy was performed due to peritonitis and multiple hepatic abscesses. At that time, he was treated with antibiotics (mainly trimethoprim-sulfamethoxazole) and interferon-g. The patient had two brothers who died due to complications of chronic granulomatous disease. In addition, both his mother and sister presented a history of discoid lupus-like lesions. Case 2: Coincidentally, his 27-year-old sister was seen in our department of dermatology 5 years before, presenting infiltrated and erythematous plaques with fine scales (Figure 3) on the right side of the nose and the left annular finger. No other cutaneous or mucous lesions were seen. She referred onset in childhood with similar lesions on sun-exposed areas that disappeared without scarring. A biopsy was performed and the results were compatible with the diagnosis of discoid lupus erythematosus (Figure 4). Direct immunofluorescence was not available. At that time, she did not mention the family history of chronic granulomatous disease. Clinical follow-up was not possible, but his brother referred that she afforded complete remission only with sun protection.
 ...
PMID:Chronic granulomatous disease: two members of a single family with different dermatologic manifestations. 1628 59

Drug eruptions are among the most common adverse drug reactions, affecting approximately 3% of hospitalised patients. Although the rate of severe cutaneous adverse reactions to medications is low, these reactions can affect anyone who takes medication, and can result in death or disability. Two general patterns can be distinguished, depending on the type of onset of these cutaneous adverse drug reactions: acute or chronic. Acute-onset events are usually rather specific cutaneous 'syndromes' that constitute emergencies and should therefore be promptly recognised and treated, while chronic-onset events often present as dermatological diseases. The challenge is therefore to recognise the drug aetiology in front of a 'classical' dermatosis such as acne, lichen or pemphigus. Therefore, clinicians should carefully evaluate the signs or symptoms of all adverse reactions thought to be drug related, and discontinue the offending agent when feasible. Erythematous drug eruptions are the most frequent and less severe acute immune drug-induced rashes, and are sometimes difficult to differentiate from viral eruptions. On the other hand, acute urticaria and angioedema are sometimes life-threatening eruptions for which a drug aetiology must be investigated. Photosensitivity, vasculitis and skin necrosis belong to the acute onset reactions, which are not always drug-induced, in contrast to fixed drug eruptions. The early recognition of acute generalised exanthematous pustulosis, DRESS (drug reaction with eosinophilia and systemic symptoms) syndrome, Stevens-Johnson syndrome and toxic epidermal necrolysis are of high importance because of the specific mechanisms involved and the different prognosis of each of these diseases. Chronic onset drug-induced disorders include pigmentary changes, drug-induced autoimmune bullous diseases, lupus, pseudo lymphoma and acneiform eruptions; these are discussed, along with specific data on drug-induced hair and nail disorders. As the disorders are numerous, the mechanisms and the drugs involved in the development of these various reactions are multiple. The list of drugs discussed in relation to the different disorders are as accurate as possible at the time of preparation of this review, but will need updating as new drugs emerge onto the market. We emphasize the clinical recognition, pathophysiology and treatment of skin, hair and nail adverse drug reactions, and the role of each doctor involved in the management of these patients in the notification of the adverse drug reaction to health authorities, using the minimal requirement for notification proposed.
 ...
PMID:Drug-induced skin, nail and hair disorders. 1797 40

In lupus erythematosus (LE), vesicles and bullae are only rarely seen. However, in some instances such efflorescences might suggest an association with distinct cutaneous diseases, including erythema multiforme, toxic epidermal necrolysis or autoimmune blistering disorders such as bullous pemphigoid, pemphigus vulgaris, and dermatitis herpetiformis Duhring. Another blistering disease that has been described in association with cutaneous and systemic LE is porphyria cutanea tarda (PCT). PCT is a metabolic disorder caused by a deficiency of the fifth enzyme in heme biosynthesis, uroporphyrinogen decarboxylase. Here, we report on a 57-year-old Caucasian woman of Dutch origin with a medical history of mild cutaneous LE who developed skin fragility, blistering skin lesions, milia, and facial hypertrichosis. Subsequent porphyrin analysis in urine and feces confirmed the suspected simultaneous manifestation of LE and PCT.
 ...
PMID:Porphyria cutanea tarda in pre-existent lupus erythematosus--is there an association? 1797 93

Validated outcome measures are essential in monitoring disease severity. Specifically in dermatology, which relies heavily on the clinical evaluation of the patient and not on laboratory values and radiographic tests, outcome measures help standardize patient care. Validated cutaneous scoring systems, much like standardized laboratory values, facilitate disease management and follow therapeutic response. Several cutaneous autoimmune dermatoses, specifically cutaneous lupus erythematosus (CLE), dermatomyositis (DM), and pemphigus vulgaris (PV), lack such outcome measures. As a result, evaluation of disease severity and patients' response to therapy over time is less reliable. Ultimately, patient care is compromised. These diseases, which are often chronic and relapsing and remitting, are also often refractory to treatment. Without outcome measures, new therapies cannot be systematically assessed in these diseases. Clinical trials that are completed without standardized outcome measures produce less reliable results. Therefore, the development of validated outcome measures in these autoimmune dermatoses is critical. However, the process of developing these tools is as important, if not more so, than their availability. This review examines the steps that should be considered when developing outcome measures, while further examining their importance in clinical practice and trials. Finally, this review more closely looks at CLE, DM, and PV and addresses the recent and ongoing progress that has been made in the development of their outcome measures.
 ...
PMID:Development of outcome measures for autoimmune dermatoses. 1798 1

Autoimmune blistering diseases are associated with autoantibodies to desmosomal (pemphigus group) or hemidesmosomal proteins (autoimmune subepidermal blistering disorders) that are essential for the structural integrity of the epidermis and dermoepidermal junction. Treatment is usually based on systemic glucocorticosteroids, which are often combined with additional immunosuppressants such as azathioprine and mycophenolate mofetil or immunomodulators including dapsone, antibiotics, and intravenous immunoglobulins. These interventions are sometimes not sufficient to induce remission and/or may be associated with intolerable adverse events. In such situations, the anti-CD20 antibody rituximab has been successfully applied in recent years. Rituximab transitorily depletes CD20-positive B lymphocytes from the circulation. It has been employed in more than 1 million patients with CD20-positive non-Hodgkin's lymphoma and severe side effects were only rarely observed. Subsequently, the B cell-modulating effect of rituximab has encouraged its use in a variety of autoimmune diseases, including more than 40 patients with pemphigus. In addition, a few patients with bullous pemphigoid, mucous membrane pemphigoid, and epidermolysis bullosa acquisita have received rituximab. In the majority of these patients, clinical remission was induced; however, serious adverse events were considerable higher compared to both patients with non-Hodgkin's lymphoma or nonbullous autoimmune disorders like lupus erythematosus, dermatomyositis, and rheumatoid arthritis.
 ...
PMID:Rituximab in treatment-resistant autoimmune blistering skin disorders. 1827 Aug 59

Rituximab is increasingly used off label for difficult-to-treat auto-immune diseases. We reviewed the main case series or clinical studies to identify the best indications of rituximab and the situations at substantial risks for adverse events. Refractory immune thrombocytopenic purpura was the main indication. However, the long term benefit-to-risk ratio of rituximab treatment before or after splenectomy is unknown. A single 375 mg/m2 infusion may be as efficacious as the classical four infusions cycle. Rituximab is the best treatment for cold agglutinin disease. In warm agglutinin auto-immune anaemia, its efficacy has essentially been reported in chronic lymphocytic leukemia (CLL) patients and in children. In CLL patients, lethal adverse events occurred in patients also receiving cyclophosphamide. Rituximab seems to have an interesting benefit-to-risk ratio in Wegener granulomatosis (excepted in granulomatous lesions), HCV-associated symptomatic cryoglobulinemia in patients unresponsive to anti-viral therapy, pemphigus and thrombotic thrombocytopenic purpura. Efficacy and safety data in lupus are difficult to interpret. Serum sickness disease is not exceptional in immune thrombocytopenic purpura (ITP), lupus and sicca syndrome patients. A substantial infectious risk has been reported in pemphigus patients and in post-renal transplant cryoglobulinemia. Double-blind randomised controlled trials and phase IV studies are mandatory in most clinical settings to confirm the overall favourable perception of rituximab benefit to risk ratio.
 ...
PMID:Rituximab off label use for difficult-to-treat auto-immune diseases: reappraisal of benefits and risks. 1827 Aug 63


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>