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)

Specialized immunological assays are required for the accurate diagnosis of bullous dermatoses such as bullous pemphigoid (BP), epidermolysis bullosa acquisita and bullous lupus erythematosus. The aim of this study was to analyse and compare the sensitivity of indirect immunofluorescence (IF) on salt-split skin and immunoblotting for the detection of circulating autoantibodies in BP. Of the BP patients selected for the study, 74/79 (94%) had circulating autoantibodies detected by at least one of the two methods. Both methods had comparable sensitivity and detected BP-specific autoantibodies in 82-85% of the patients. Because 20% of the patients were found to be positive by only one of the methods, both methods should be used in the diagnosis of BP. Indirect IF on salt-split skin is easier to perform and is preferable in routine analysis, but Western blotting may be used as a complementary assay with sera showing no reactivity on salt-split skin.
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PMID:Serological diagnosis of bullous pemphigoid (BP): comparison of the sensitivity of indirect immunofluorescence on salt-split skin to immunoblotting. 155 99

We examined the incidence of epidermolysis bullosa acquisita in patients with circulating basement membrane zone antibodies. Serum samples from 100 sequential patients with basement membrane zone antibodies were tested by indirect immunofluorescence against 1 mol/L sodium chloride split skin and by Western immunoblot against epidermal and dermal extracts of skin. Ninety-two (92%) serum samples stained only the epidermal side of split skin, 5 (5%) stained only the dermal side, and 3 (3%) stained both sides. Four of the 5 serum samples with dermal staining but none of the serum samples with epidermal or combined staining reacted with the 290-kd epidermolysis bullosa acquisita antigen by Western immunoblot. These results indicate that approximately 5% of unselected patients with basement membrane zone antibodies have epidermolysis bullosa acquisita or bullous lupus erythematosus rather than bullous pemphigoid.
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PMID:Epidermolysis bullosa acquisita. Incidence in patients with basement membrane zone antibodies. 222 45

There exists a multitude of systemic diseases which exhibit consequential oral manifestations. Dermatologic disorders frequently involve intraoral and perioral tissues which dental practitioners should be familiar with. A compilation of disorders including; bullous diseases (such as Pemphigus Vulgaris, Erythema Multiforme, Pemphigoid, Dermatitis Herpetiformis, Epidermolysis Bullosa, and Darier's disease), as well as non bullous diseases (such as Lichen Planus, Psoriasis, Lupus erythematosus, Scleroderma, and Perioral Dermatitis); and their oral manifestations are presented in order to facilitate their early diagnosis and subsequent treatment.
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PMID:[Oral manifestations of skin diseases]. 222

In most cases of epidermolysis bullosa acquisita that occur in patients with systemic lupus erythematosus, the diagnosis of systemic lupus erythematosus is made before the development of blistering. We observed three patients with well-documented epidermolysis bullosa acquisita that developed several years before the onset of systemic lupus erythematosus. One patient was producing anti-U1RNP autoantibodies at the time epidermolysis bullosa acquisita was diagnosed, and all five produced this antibody during the systemic lupus erythematosus phase of their illness. In addition, in all five cases of epidermolysis bullosa acquisita with systemic lupus erythematosus antibodies to double-stranded DNA ultimately developed, and severe systemic lupus erythematosus and lupus nephritis developed in four patients. Sera from 15 other patients with epidermolysis bullosa acquisita without overt systemic lupus erythematosus were analyzed for systemic lupus erythematosus-related autoantibodies. Four patients were found to have at least one such autoantibody. These findings further document an association between epidermolysis bullosa acquisita and systemic lupus erythematosus and suggest that patients with systemic lupus erythematosus who present with epidermolysis bullosa acquisita may represent a subset of lupus erythematosus that puts the patient at increased risk for the development of more severe systemic illness. Patients presenting with epidermolysis bullosa acquisita, especially those who are black or Hispanic, should be monitored for the development of potentially life-threatening systemic lupus erythematosus.
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PMID:Epidermolysis bullosa acquisita preceding the development of systemic lupus erythematosus. 231 19

Skin affected by a burn cancer is scarred, ulcerated, and often appears as erythema ab igne clinically in adjacent skin. The latent period in burn scar malignancy is much longer for SCC than BCC. Malignant melanoma and various sarcomas are reported to arise in burn scars, too. The other extreme on the temperature scale can less often result in enough permanent acral damage that poor wound healing may eventually result in cancer, usually SCC. About 1% of patients with chronic osteomyelitis develop cancer, usually SCC in sinus tracts. As with tumors arising in burn scars and chronic leg ulcers of varied etiology, black patients are disproportionately overrepresented in osteomyelitic malignancy. In nearly all of the patients with radiation-induced skin cancer, concomitant radiodermatitis is present. As with burn scar and osteomyelitic cancer, x-ray related cancer has a long latent period. Similar to burn scar cancer, SCC predominates in osteomyelitis and occurs on the extremities. BCC, when it arises, is more common on the face and neck in burn- and radiation-induced tumors. Multiple tumors are frequent as is recurrence in x-ray malignancy. Mortality is high: one out of three to four patients with burn scar, osteomyelitic, and radiation cancer die of dermatosis-related malignancy. Recently, radioactivity-contaminated gold rings have been implicated in causing SCC. Carcinoma tends to occur in irradiated benign dermatoses whereas sarcomas tend to complicate irradiated malignancies. Stasis ulceration and anogenital fistulae may rarely lead to cancer, SCC in the former and adenocarcinoma in the latter. SCC can rarely develop in four related conditions (acne conglobata, dissecting perifolliculitis of the scalp, hidradenitis suppurativa, and pilonidal sinus) after a lengthy latent period; prognosis is poor with a high metastatic rate. A whole host of chronic cutaneous infections can lead to malignancy occasionally; these include lupus vulgaris, lymphogranuloma veverum, granuloma inguinale, leprosy, actinomycosis, and candidiasis. BCC more than SCC is known to complicate smallpox vaccination sites. Certain erosive and/or scarring dermatoses other than those mentioned above can be unusually affected by secondary malignancy. Discoid lupus erythematosus lesions often subjected to the carcinogenic effects of sunlight can degenerate into SCC in patients with either light or dark skin. In acrodermatis chronica atrophicans, a condition not often seen in the United States, the involved skin, particularly of the lower extremities, is susceptible to SCC, lymphoma, and BCC. Epidermolysis bullosa, especially the recessive dystrophic variant, can be complicated by SCC on affected mucous membrane and acral skin.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Cancer complicating chronic ulcerative and scarifying mucocutaneous disorders. 307 55

Diseases in which involvement of the nails may be helpful in the diagnosis of dermatologic disease elsewhere include psoriasis, lichen planus, Darier's disease, alopecia areata and totalis, keratotic scabies, scleroderma, and lupus erythematosus and dermatomyositis. Dermatologic diseases in which involvement of the nail commonly occurs but is relatively nonspecific and not diagnostic include dermatitis, lichen striatus, parakeratosis pustulosa, pityriasis rubra pilaris, acrokeratosis paraneoplastica, pemphigus vulgaris, epidermolysis bullosa, porokeratosis of Mibelli, and acanthosis nigricans. These entities are described and treatments summarized as appropriate.
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PMID:Dermatologic diseases of the nail unit other than psoriasis and lichen planus. 383 May 3

An artifact, consisting of hydropic degeneration of the basal cells and subepidermal bulla formation, is described in skin-punch biopsy specimens immersed in normal saline. The histologic appearance and ultrastructural features of this artifact are discussed. Proper handling of skin specimens intended for immunofluorescent studies is stressed. Similarities are noted between the histologic and ultrastructural findings of this artifact and of epidermolysis bullosa simplex and pathologic hydropic degeneration, as is seen in lupus erythematosus.
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PMID:Artifactual hydropic degeneration in skin biopsy specimens immersed in saline: a light and electron microscopic study. 634 24

Globular deposits of immunoglobulins in the papillary dermis have been reported to occur in certain dermatoses, particularly in lichen planus. The clinical correlation of these deposits in 52 skin biopsy specimens reviewed by light and fluorescent microscopy was studied. These cases included five of lichen planus, 24 of lupus erythematosus or related diseases, four of dermatitis herpetiformis, three of drug eruption; two each of bullous pemphigoid, erythema nodosum, porphyria cutanea tarda; one each of vitiligo, pyoderma gangrenosum, neurodermatitis, erythema multiforme, granuloma annulare, vasculitis, epidermolysis bullosa simplex, Rothmund-Thompson syndrome, and four of unspecified dermatoses. Using an arbitrary scale of 1-4 based on the frequency of deposits, 3+ and 4+ deposits were identified in all five cases of lichen planus, as well as in six cases of lupus erythematosus, one of drug eruption, one of bullous pemphigoid, one of erythema nodosum, the vitiligo, vasculitis, and Rothmund-Thompson syndrome cases, and two cases of unspecified dermatoses; other cases showed only 1+ and 2+ deposits. In all five cases of lichen planus, the deposits contained IgM and C3, and in addition, IgA was present in four, IgG and fibrinogen in three. Among non-lichen planus cases, C3 was detected in 11 of 49, and fibrinogen in only four of 49. These findings indicate that globular deposits of Ig in the dermis, though suggestive, are not pathognomonic of lichen planus.
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PMID:Globular deposits of immunoglobulins and complement in the papillary dermis. Clinical significance. 736 75

Autoantibodies to type VII collagen are associated with the blistering diseases epidermolysis bullosa acquisita and bullous systemic lupus erythematosus. We showed previously that these autoantibodies recognize epitopes within the noncollagenous (NC1) region of type VII collagen. That region is composed of fibronectin type III homology units that may contribute to intermolecular cross-linking and basement membrane adhesion functions of type VII collagen. In this study, we defined the specific amino acid sequences recognized by these autoantibodies. By fusion protein analysis, sera from patients with epidermolysis bullosa acquisita and bullous lupus were found to react with two regions within the fourth (E-1) and eighth (E-2) fibronectin homology repeats, each consisting of approximately 100 amino acids. Affinity purification studies showed E-1 and E-2 to be independent and non-cross-reactive epitope regions. These regions were probed further by enzyme-linked immunosorbent assay analysis of overlapping octapeptide sets derived from the amino acid sequences of E-1 and E-2. The results showed two reactive, closely associated octapeptide sequences within each region, both lying in amphipathic portions of fibronectin type III homology repeats. These studies identify short peptide sequences within the NC1 domain of type VII collagen that are targeted independently by autoantibodies. These sequences may play a direct role in determining the properties of type VII collagen that influence adhesion between this molecule and other basement membrane proteins, and their alteration by antibody binding may be the immunopathogenic event underlying epidermolysis bullosa acquisita and bullous lupus.
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PMID:Immunodominant autoepitopes of type VII collagen are short, paired peptide sequences within the fibronectin type III homology region of the noncollagenous (NC1) domain. 753 Feb 71

Epidermolysis bullosa acquisita may be associated with various systemic diseases, including systemic lupus erythematosus. We describe the clinical and immunological findings in a 38-year-old women with epidermolysis bullosa acquisita and systemic lupus erythematosus. The epidermolysis bullosa acquisita preceded a dramatic flare of systemic lupus erythematosus and fatal cerebral vasculitis. If serologic evidence of lupus erythematosus develops during the course of epidermolysis bullosa acquisita, a thorough investigation is warranted to rule out potentially life-threatening systemic lupus erythematosus.
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PMID:Fatal vascular involvement in systemic lupus erythematosus following epidermolysis bullosa acquisita. 760 45


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