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Management of active blisters Most blisters in an EB patient will spontaneously heal if given sufficient time i.e. days to weeks ; . Unfortunately this conservative approach may be associated with considerable pain. Blisters on the soles of the feet, for example, may prevent an affected individual from walking or even standing. It is generally preferable, therefore, that large blisters or any blisters that are painful or interfere with daily activities be sterilely drained. Drainage of blisters on or near the face can usually be avoided, as can any which arise within the oral cavity. The preferred approach is to first gently cleanse the blister's surface with isopropyl alcohol, followed by its lancing with a small sterile needle which can be prescribed by the patient's physician ; . If the latter cannot be obtained or afforded, then a reasonable alternative is to use a fine, clean, sewing needle which has been kept immersed in isopropyl alcohol and then briefly sterilized by careful heating over a lit match or flame until its tip is yellow. It can then be used as soon as it is again cool. Once the punctured blister has been drained of its contents by the gentle application of pressure with a clean gauze pad, a tiny dab of a mild antibiotic ointment should be placed over the puncture site, followed by application of a sterile dressing, so as to reduce the risk of secondary infection developing within the puncture site. If necessary, a blister can be drained numerous times, if it repeatedly re-fills with fluid. Pain, itching and EB Patients with EB frequently experience painful blisters and open sores. When necessary, the patient's physician may prescribe brief courses of appropriate analgesics, although the chronic use of codeine-containing medications, as well as other narcotics, should be avoided since 1 ; their use may lead to dependency, and 2 ; chronic use may cause or worsen pre-existing constipation, which is also a common complication of the more severe forms of inherited EB. Itching may be a sign of secondary infection, although it can also occur in its absence. Systemic antihistamines may be helpful, but should be used only with the approval of a physician. Cancer surveillance and EB Patients with one of the more severe forms of inherited EB, so-called recessive dystrophic EB RDEB ; , are at considerable risk of developing one or more skin cancers squamous cell carcinomas, most commonly; malignant melanoma, rarely ; during their lifetimes. Unfortunately, these tumors, which tend to arise in chronically, non-healing, skin sores, oftentimes later spread internally, eventuating in death. Although not a problem in infancy or early childhood, they can arise as early as about age 12. By age 35, for example, approximately half of all patients with the Hallopeau-Siemens subtype of RDEB will have developed at least one squamous cell carcinoma. Therefore, it is very important that all patients with RDEB have at least yearly examination of all skin areas, even those which are chronically covered in bandages, beginning during early adolescence. Furthermore, any skin sore which is not healing like other lesions should be immediately brought to the attention of the patient's dermatologist and biopsied to exclude the presence of early malignant transformation. Resources A comprehensive monograph on EB has recently been published * . Copies may be purchased via the Internet through either the publisher Johns Hopkins University Press ; or any major on-line bookseller. Epidermolysis Bullosa. Clinical, Epidemiologic, and Laboratory Advances and the Findings of the National Epidermolysis Bullosa Registry. edited by Fine J-D, Bauer EA, McGuire J, and Moshell A. Johns Hopkins University Press, Baltimore, May 1999, 490 pages. You may contact DebRA of America with questions: 866 ; 332-7276 nurse debra ; debra.
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Evidence of variability in PR3- and MPO-ANCA ELISA kits [67] underscores this concern. The common practice of heating 56C ; sera before use to inactivate viruses and complement may need to be avoided, because this manipulation has been shown, in an as yet unexplained way, to yield "false positive" results with sera from healthy donors [66]. It is discussed that natural autoantibodies that function as scavenger molecules are responsible for this phenomenon. We believe that a more complete and reliable assessment of BPI-ANCA requires testing sera from patients with ANCA-associated diseases for BPI-ANCA regardless of IIF results and standardization of BPI-ANCA ELISA protocols to minimize procedural differences. A prospective multi-center study to determine prevalence, prognostic, and immunodiagnostic value of BPI-ANCA should be started, as was done for PR3-ANCA and MPOANCA [68]. In the case of ANCA-associated vasculitides, the presence of cANCA in IIF and PR3-ANCA by ELISA is highly sensitive and specific for the diagnosis of Wegener's granulomatosis [9]. For the reasons described above, it is not yet possible to assess the likely immunodiagnostic significance of BPI-ANCA in most settings in which it has been detected. Perhaps an exception is in inflammatory glomerular diseases where, of nearly 400 patients studied, 20 30% contain BPI-ANCA [43]. No association of BPI-ANCA with a particular type of renal injury was apparent. In contrast, in patients with inflammatory bowel disease, primary sclerosing cholangitis, and CF, which are BPI-ANCA-positive, a higher inflammatory activity and greater organ damage are generally seen [44 52, 59, 65]. In addition, in CF, BPI-ANCA titers were related inversely to lung function and chest radiograph score and highest in patients colonized by Pseudomonas [49, 51]. However, all these studies suffer from the small numbers of patients analyzed, making comparison of disease activity, time of testing, or associated conditions e.g., infection ; problematic. Prospective longitudinal studies with defined patient groups are needed for more comprehensive evaluation. In diseases not associated with ANCA such as leukocytoclastic cutaneous vasculits or bronchectasis with Pseudomonas infection ; , only single patients with BPI-ANCA have been shown [61, 64]. Yet, there seem to be common features shared by individuals with BPI-ANCA: CF, IBD, PSC, and also in -1-anti-trypsin deficiency with bronchiectasis and Pseudomonas infection includes a setting where exposure to Gram-negative bacteria and cell-free LPS is appreciable and sometimes chronic e.g., CF ; . In these conditions, a neutrophilrich inflammatory response can be observed that is likely associated with extracellular mobilization of BPI and subsequent formation of BPI-LPS complexes. Recent observations suggest that BPI-LPS complexes are targeted to monocytes playing an important role in antigen presentation unpublished results ; . Thus, it can be hypothesized that BPI-LPS complexes or degraded neutrophils, after being delivered to monocytes [69], are processed, and BPI domains--as well as other granule-protein domains--may be presented on the cell surface giving way to the generation of BPI autoantibodies. Given the apparent association with Gram-negative bacterial infections, the possibility that BPI-ANCA represent, at least in part, cross-reactive antibodies induced by bacterial LPS-binding proteins should also be considered [70, 71] and methoxsalen.

Absolutely frightening, horrifying." A majority of those who walked through Intersection exited with a fear they will never forget, a fear that will serve as a reminder for them to seek regular, comprehensive eye exams. As people entered Intersection, they were greeted by employees of The Glaucoma Foundation and offered free literature. The Foundation also distributed free give-aways with printed statements on them reminding everyone to "Get Your Eyes Checked" and to call 1-800-GLAUCOMA with questions. The give-aways alternated daily between post-it pads and key chains with small flashlights attached. At the exit of the exhibit, staff was also available to answer questions about glaucoma and treatments to prevent related blindness. More than 2, 000 people experienced what it was like to be blind as they walked through the virtual reality of Intersection. The Glaucoma Foundation was also successful in educating people about glaucoma through Health Expo's Speakers' Corner. Dr. Gregory Harmon, Chief of Glaucoma at New York Hospital-Cornell Medical Center, and a member of our Board of Directors, held a roundtable discussion on Monday, September 15. Edith Marks and Rita Montauredes, authors of the newly published book Coping with Glaucoma see page 3 ; , were present to discuss glaucoma and to sign books at the Barnes & Noble Bookstore Exhibit on September 14. Readers' Note: The next Health Expo fair is scheduled for September 23-27, 1998, in Dallas, Texas. 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