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

The photoaggravated dermatoses are diverse diseases adversely affected by sunlight or artificial ultraviolet (UV) exposure. The role of UVR is often well-recognized, as in lupus erythematosus (LE) and bullous pemphigoid (BP). Immunologic theories for the cause of the photosensitivity in lupus erythematosus are the production of UV-DNA antibodies with subsequent immune complex formation, a partial defect in DNA repair following UV exposure, and the release or stimulation of potent cutaneous immunologic mediators. However, mechanisms are still not fully elucidated. Photosensitivity has also been described in erythema multiforme, actinic lichen planus, viral exanthems, cutaneous T-cell lymphoma, eczema, and psoriasis. Treatment is restriction of light exposure, use of high protection sunscreens, and treatment of the underlying disorder.
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PMID:The photoaggravated dermatoses. 220 46

Biopsy specimens from 185 patients with 52 different skin disorders were investigated by indirect immunofluorescence staining for the presence of HLA-DR bearing keratinocytes and their association with an underlying inflammatory infiltrate and in particular with activated (HLA-DR-positive, Leu-4-positive) T lymphocytes. HLA-DR expression on keratinocytes was demonstrated in 38 dermatoses, including lymphocytic vasculitis, lupus erythematosus, morphea, vitiligo, lichen planus, cutaneous T-cell lymphoma, various infectious dermatoses, allergic contact dermatitis, granulomatous dermatoses, Sweet's syndrome, lichen sclerosus and erythema nodosum. In 27 of these this had not previously been reported. Occurrence of HLA-DR on keratinocytes was invariably linked to the presence of a lymphocytic infiltrate containing numerous activated T-cells (Leu-4 +, HLA-DR +) whereas such infiltrates were not accompanied by HLA-DR expression on keratinocytes in all the dermatoses investigated, as in pseudolymphoma and erythema anulare centrifugum. However, HLA-DR positive keratinocytes were consistently absent in skin disorders lacking any significant lymphocytic infiltration (e.g. leukocytoclastic vasculitis, bullous autoimmune dermatoses, genodermatoses and mastocytosis). Although it has been suggested that HLA-DR-positive keratinocytes are involved in various immune responses of the skin, their exact functional significance is, as yet, unknown.
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PMID:HLA-DR expression on keratinocytes is a common feature of diseased skin. 242 56

Beyond routine hematoxylin-eosin histologic examinations, specialized diagnostic techniques allow examination of biopsy material for subtle morphological and functional alterations. Morphological analytic techniques (1-micron section analysis, transmission electron microscopy, x-ray probe microanalysis, and digital image analysis) and functional analytic techniques (immunofluorescence, immunohistochemistry, and molecular biologic techniques) are valuable diagnostic tools. These techniques have applications in evaluating cutaneous T-cell lymphoma and atypical lymphocytic infiltrates, vesiculobullous disorders, lupus erythematosus and collagen vascular diseases, vasculitis, poorly differentiated tumors, storage diseases, and infections.
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PMID:Special techniques in dermatology. 266 12

We reviewed 62 cases (26 men and 36 women, median age 55 years) of previously diagnosed lichenoid dermatitis and correlated the histologic and immunodermatologic findings with the clinical diagnosis and course. The clinical diagnostic groups were contact dermatitis (6 cases), drug eruption (14), lupus erythematosus (6), lichen planus (3), cutaneous T cell lymphoma (3), chronic dermatitis or neurodermatitis (19), and miscellaneous dermatologic disorders (11). With the use of individual histologic features, a correlation with specific clinical conditions was possible, and the clinical groups of dermatitis, drug eruption, lupus erythematosus or lichen planus, and T cell infiltration could be selected histologically. Direct immunofluorescence studies were of most help in the diagnosis of lupus erythematosus and atypical lichen planus, but these studies should be performed in all cases involving lichenoid inflammation. Although previously the nonspecific term lichenoid dermatitis was used to classify the histologic features in these cases, we found that by careful correlation of histologic, immunodermatologic, and clinical features, we were often able to provide a specific diagnosis.
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PMID:Lichenoid dermatitis: a clinicopathologic and immunopathologic review of sixty-two cases. 276 78

Photopheresis is a relatively safe and technically simple modality for immunomodulation. The extracorporeal activation of a drug with UVA constitutes a novel form of drug delivery. The usefulness of this system in erythrodermic CTCL is well established. Immunomodulating therapy can thus be conducted outside the host, where controlled conditions permit cellular manipulations not possible in the intact patient. This system is an excellent paradigm for future immunomodulating therapies in diseases such as pemphigus vulgaris and lupus erythematosus.
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PMID:Photopheresis for T cell mediated diseases. 315 22

In the present study, an analysis was made of the expression pattern of thrombospondin-1 (TSP1) and its receptor (CD36) in skin biopsies obtained from healthy volunteers and from patients with lichen planus, lupus erythematosus, cutaneous T-cell lymphoma and psoriasis vulgaris. Using monoclonal antibodies against TSP1 in biopsies from the healthy volunteers and from both clinically involved and uninvolved skin of the patients, a specific peroxidase-positive reaction was detected around the sweat glands in the dermis. In all cases investigated, the CD36-positive lesional keratinocytes remained TSP1-negative. These findings favour the hypothesis that CD36-positive keratinocytes might have some functional relevance via oxidized low-density lipoprotein and/or collagen fibrils, without any connection with TSP1.
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PMID:Expression of thrombospondin-1 (TSP1) and its receptor (CD36) in healthy and diseased human skin. 752 82

Extracorporeal photophoresis (ECP), a therapeutic modality that has been under investigation for some years, is based on separation of a leucocyte/lymphocyte-enriched cell fraction from the peripheral blood, extracorporeal treatment of the cells with 8-MOP/UVA and subsequent reinfusion of the cells in the patient. Its main effects seem to consist in changes to the immunologic behaviour of the photoinactivated/modulated cells. The immune response of the host is obviously stimulated by this treatment. ECP is normally performed for 4 h per day on 2 consecutive days every 4 weeks. The treatment is well tolerated and causes few side effects. In our department, 1210 ECP treatments were administered to 41 patients between 1990 and 1994 and a preliminary evaluation was performed. These patients included 21 with cutaneous T-cell lymphoma (CTCL), 10 with progressive systemic scleroderma, 4 with chronic graft-versus-host disease and 1 each with pemphigus vulgaris, epidermolysis bullosa acquisita, lupus erythematosus and cutaneous mucinosis. Patients with erythroderma and preserved immunocompetence achieved the best responses of all patients with CTCL. A treatment combining ECP with rIFN-alpha, PUVA and/or radiation was also successful in patients with tumour-stage CTCL and lymph node involvement. Progressive systemic scleroderma responded in more than 50% of our cases. Treatment results were impressive in 4 patients with chronic graft-versus-host disease presenting with sclerodermatous and lichenoid changes of the skin and mucous membranes. A clear improvement was also observed in the patient with pemphigus vulgaris refractory to standard therapies and in another patient with scleromyxoedema (Arndt-Gottron syndrome). The effectiveness of ECP seems to be quite well established in CTCL, but remains to be examined in autoimmune dermatoses. ECP is an attractive addition to the dermatological therapies available but our experience is still preliminary.
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PMID:[Therapeutic experiences with extracorporeal photopheresis. Technical procedure, follow-up and clinical outcome in 31 skin diseases]. 886 55

The antiphospholipid syndrome is an acquired multisystem disorder of hypercoagulation, which may be primary or secondary to underlying diseases. Serologic markers for the syndrome are the lupus anticoagulant and anticardiolipin antibodies. Clinical features include recurrent thrombotic events (arterial or venous), repeated fetal loss, and thrombocytopenia. Cutaneous manifestations may occur as the first sign of antiphospholipid syndrome. These include livedo reticularis, necrotizing vasculitis, livedoid vasculitis, thrombophlebitis, cutaneous ulceration and necrosis, erythematous macules, purpura, ecchymoses, painful skin nodules, and subungual splinter hemorrhages. Antiphospholipid syndrome may also be associated rarely with anetoderma, discoid lupus erythematosus, cutaneous T-cell lymphoma, or disorders that closely resemble Sneddon or Degos syndromes. Noninflammatory vascular thrombosis is the most frequent histopathologic feature observed. Prophylaxis and treatment of thrombosis in patients with antiphospholipid syndrome relies principally on anticoagulant and antiplatelet agents.
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PMID:Antiphospholipid syndrome and the skin. 920 65

Photopheresis is an extracorporeal form of photochemotherapy with 8-methoxypsoralen (8-MOP) and ultraviolet A (UVA) radiation. Photopheresis is used for the management of T-cell-mediated diseases, and such treatment leads to the induction of antigen-specific immune suppression directed to the pathogenic clone of T cells. Photopheresis is used to treat a wide variety of diseases--such as cutaneous T-cell lymphoma, systemic sclerosis; rheumatoid arthritis, lupus erythematosus--and is also successfully applied in the suppression of graft rejection. In addition to the clinical achievements, attention will be paid to results from animal studies. An important outcome of these studies is that photopheresis can be used to treat airway hyperreactivity. Furthermore, it was shown that the therapeutic strategy can be changed drastically: the presence of plasma during irradiation should be avoided and the amount of blood that must be treated to obtain the desired antigen-specific immunosuppression can be greatly decreased. Also, results from cellular experiments are discussed. An example of this is the increase in the major histocompatibility complex expression on the surface of cells found after treatment. The mechanism that underlies photopheresis has not yet been elucidated, but progress has been made. The following related points will be reviewed: models for investigation; and mechanistic aspects, with the emphasis on cellular biomacromolecules and on photosensitizers (drugs) other than 8-MOP.
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PMID:Clinical and mechanistic aspects of photopheresis. 922 56

We present 20 patients in whom drug therapy was associated with interstitial histiocytic infiltrates with variable degeneration of collagen and elastic fibers mimicking early lesions of granuloma annulare (GA). Most patients had a reproducible clinical presentation comprising erythematous-to-violaceous, nonpruritic plaques, often with an annular pattern, predominantly involving inner aspects of the arms, medial thighs and intertriginous areas. The most frequent clinical differential diagnoses included cutaneous T cell lymphoma, erythema annulare centrifigum (EAC), GA, and lupus erythematosus. A drug reaction was suspected in only 3 cases. The implicated drug classes included calcium channel blockers, angiotensin converting enzyme inhibitors, beta-blockers, lipid-lowering agents, antihistamines, anticonvulsants and antidepressants. Patients were often on two or more of these drugs; all have been associated with pseudolymphomatous infiltrates of the skin, the presumptive basis of which is iatrogenic pertubation of immune function. The defining histomorphology was diffuse infiltration of the interstitium by lymphocytes and histiocytes with piecemeal fragmentation of collagen and elastic fibers in concert with a vacuolar interface dermatitis. Ten cases showed intermediate and transformed lymphocytes with hyperchromatic convoluted nuclei disposed interstitially within the dermis or along the dermoepiderma junction with variable epidermotropism. In the 15 patients who discontinued the implicated drug, lesional resolution occurred. We propose the designations interstitial granulomatous drug reaction for this novel cutaneous reaction pattern.
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PMID:The interstitial granulomatous drug reaction: a distinctive clinical and pathological entity. 952 95


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