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Biomedical subjects

Victoria P Werth

Publications and source records attributed to Victoria P Werth.

At least 19 recordsLinked to original sources
PubMed2006

Cross-sectional study of bisphosphonate use in dermatology patients receiving long-term oral corticosteroid therapy.

OBJECTIVE: To examine whether patients had received bisphosphonates at the beginning of planned long-term glucocorticoid therapy, which is recommended by the guidelines from the American College of Rheumatology to prevent glucocorticoid-induced osteoporosis, prior to referral to a tertiary dermatology clinic. DESIGN: Cross-sectional study. SETTING: Tertiary referral center. PATIENTS: We reviewed 35 patients from an established cohort of patients referred with chronic skin diseases that require prolonged glucocorticoid use. MAIN OUTCOME MEASURE: The use of osteoporosis prophylaxis was determined by medical chart review and communication with patients. RESULTS: Of 35 patients, 28 (80%) were not receiving any bisphosphonates at referral. These patients began glucocorticoid therapy 17 months (median, 6 months; range, 1-102 months) prior to referral. The proportion of patients treated with bisphosphonates in our cohort did not change after the guidelines of the American College of Rheumatology were published. CONCLUSIONS: For patients of non-child-bearing potential with dermatological diseases in which prolonged oral corticosteroid treatment is anticipated, bisphosphonates should be prescribed concomitantly with the initiation of glucocorticoid therapy.

Absorptiometry, Photon↗
PubMed2006

Improvement in dermatomyositis rash associated with the use of antiestrogen medication.

BACKGROUND: Dermatomyositis (DM) is an autoimmune disorder that occurs more often in women than men and causes highly symptomatic and inflammatory cutaneous and proximal muscle disease. Corticosteroids have been the treatment of choice for myositis in DM, and antimalarial agents for the skin disease of DM, with methotrexate sodium, azathioprine, mycophenolate mofetil, cyclosporine, and intravenous immunoglobulin used as steroid-sparing agents. Recently, reports supporting a role for anti-tumor necrosis factor alpha (TNF-alpha) therapy in the treatment of DM have emerged. OBSERVATIONS: We describe 2 women who experienced an improvement in their DM-associated skin eruptions while taking antiestrogen medication. The first patient was taking tamoxifen, a selective estrogen receptor modulator that has been found to have anti-TNF-alpha properties. The second was taking anastrozole, an aromatase inhibitor. When tamoxifen therapy was discontinued after 4 years of use in the first patient, her DM rash worsened and remained difficult to control with conventional immunosuppressant medication. CONCLUSIONS: With the limited number of therapies available to manage DM skin eruptions, the discovery of novel agents effective in treating this disease is vital. Using antiestrogen medication in women with DM may result in a significant improvement in their rash, possibly via the inhibition of TNF-alpha production by immune or other cells. Further investigation into the use of antiestrogen therapy in DM is merited to evaluate long-term risks and benefits.

Adult↗
PubMed2006

Skin disorders with arthritis.

Many inflammatory, metabolic and infectious diseases affect the skin and joints. Most of these, such as rheumatoid arthritis and systemic lupus erythaematosus, are considered to be rheumatic conditions with secondary skin involvement. However, several primary cutaneous diseases are associated with arthritis and may even present with joint symptoms prior to cutaneous lesions. Common skin disorders, such as acne and psoriasis, have well-known musculoskeletal manifestations. Other less common conditions, such as dermatomyositis, multicentric reticulohistiocytosis, pyoderma gangrenosum, Sweet's syndrome and various cutaneous vasculitides, also have frequent joint involvement. This review will discuss the clinical presentation, both cutaneous and musculoskeletal, diagnosis and management of these disorders.

Acne Vulgaris↗
PubMed2006

What is new in the treatment of steroid-induced osteoporosis?

Glucocorticoid-induced osteoporosis (GIOP) is a serious complication resulting from long-term steroid treatment. In addition to several nonpharmacologic therapies recommended by the American College of Rheumatology, various pharmacologic therapies, such as calcium, vitamin D, hormone-replacement therapy, calcitonin, and bisphosphonates, can be used to prevent and/or treat GIOP. Bisphosphonates, which are potent inhibitors of bone resorption, are considered the most effective and first-line agents for increasing bone mineral density and decreasing the risk of fracture. Human parathyroid hormone has emerged as a promising agent for the treatment of severe GIOP when used alone or in combination with a bisphosphonate.

Diphosphonates↗
PubMed2006

Pemphigus: a treatment update.

Pemphigus is a group of rare autoimmune mucocutaneous bullous diseases with potential significant morbidity and mortality. The two main subtypes are pemphigus vulgaris (PV) and pemphigus foliaceus (PF). Systemic corticosteroid use and other advances in management have dramatically decreased the mortality rate for pemphigus. At present, the primary cause of morbidity and mortality is complications from treatment. Thus, the goal of pemphigus management is to induce and maintain remission with the lowest possible doses of medication and with the fewest side effects. Although our scientific knowledge of pemphigus is advancing and our treatment options are expanding, there are still very few randomized, controlled studies to evaluate the true effectiveness of the available therapies. Here we review the available treatment options and novel therapies for pemphigus and the supporting data.

Animals↗
PubMed2006

Failure of physiologic doses of pure UVA or UVB to induce lesions in photosensitive cutaneous lupus erythematosus: implications for phototesting.

BACKGROUND: Phototesting studies in cutaneous lupus erythematosus have yielded variable results, with most trials reporting photo-induction of lesions by both UVA and UVB in substantial numbers of patients. OBJECTIVES: To determine the minimal erythema dose in patients with subacute cutaneous lupus erythematosus (SCLE) and controls. PATIENTS/METHODS: We phototested nine patients with SCLE and 14 skin type-matched controls, using repetitive dosing of UVA1 and UVB, but with filters that removed most of the shorter UVC and longer infrared and visible light. In addition, DNA was isolated from anticoagulated blood to genotype the TNF-alpha 308 region in each patient and control. RESULTS: We were unable to demonstrate a difference in minimal erythema dose (MED) between patients and controls, or any correlation of MED with either TNF genotype or systemic drug therapy for SCLE. In addition, no SCLE skin lesions were induced in the nine patients with either UVA or UVB, and one patient cleared a skin lesion after low-dose UVA1 irradiation. CONCLUSIONS: The potential role of wavelengths outside the UVA and UVB range in the photo-induction of cutaneous lupus skin lesions needs to be investigated, and there is a need to standardize phototesting equipment and procedures for patients with cutaneous lupus erythematous.

Case-Control Studies↗
PubMed2006

Management of cutaneous dermatomyositis: current therapeutic options.

Dermatomyositis is an idiopathic inflammatory myopathy with characteristic cutaneous manifestations and proximal muscle weakness. Patients with this condition frequently require treatment for muscular, systemic, and cutaneous involvement; however, the cutaneous symptoms often cause significant morbidity and frustration to patients. The skin lesions of dermatomyositis often persist or recur after treatment of myositis, and there are subsets of patients who have significant cutaneous disease with little or no muscle involvement. Only anecdotal evidence is available for cutaneous treatment, and the approach to therapy is guided by disease severity and refractoriness. First-line therapy for localized disease begins with topical agents such as antipruritics and/or corticosteroids, while widespread disease often requires initial systemic therapy. Antimalarials are generally used for significant skin disease, followed by other anti-inflammatory agents, systemic corticosteroids, corticosteroid-sparing immunosuppressants and, recently, biologics and intravenous immunoglobulin. While there is a recognized absence of randomized prospective clinical trials examining the optimal monotherapy and combination treatment for the cutaneous manifestations of dermatomyositis, we have tried to provide a review of the literature and a systematic approach to dealing with these often refractory and debilitating symptoms.

Administration, Topical↗
PubMed2005

Clinical manifestations of cutaneous lupus erythematosus.

The skin findings seen in lupus erythematosus can present with either lupus-specific or lupus-nonspecific findings, with lupus-specific skin disease showing findings histopathologically distinct for cutaneous lupus erythematosus. Lupus-specific skin diseases include chronic cutaneous, subacute cutaneous, and acute cutaneous lupus erythematosus. The types of skin lesions in each group are clinically distinct and recognizing the specific subsets helps in prognosticating the likelihood of underlying systemic lupus. A number of medications are associated with cutaneous lupus, in particular with subacute cutaneous lupus erythematosus. Lupus nonspecific skin lesions are not histopathologically distinct for cutaneous lupus and/or may be seen as a feature of another disease process. Nonspecific disease-related skin lesions are frequently seen in patients with SLE, usually in the active phase of the disease. The current ACR classification criteria for SLE include four somewhat overlapping dermatologic criteria, butterfly rash, discoid lupus, photosensitivity, and oral ulcers and thus patients can be classified as having SLE with only skin manifestations.

Acute Disease↗
PubMed2005

Dapsone as a glucocorticoid-sparing agent in maintenance-phase pemphigus vulgaris.

OBJECTIVE: To determine the effect of dapsone on glucocorticoid-dependent patients with active or maintenance-phase pemphigus vulgaris. DESIGN: Retrospective study of consecutive patients treated with dapsone. SETTING: University of Pennsylvania, Philadelphia (a tertiary referral hospital). Patients We observed 9 consecutive adult patients with pemphigus vulgaris being treated with immunosuppressants who were unable to taper prednisone use without abrupt worsening of their disease.Interventions Dapsone treatment added to prednisone and other immunosuppressive therapy. Main Outcome Measure Steroid dosage. RESULTS: All patients were unable to taper their steroid dose during the 3 months prior to the initiation of dapsone therapy or had active disease that was not well controlled by prednisone prior to dapsone treatment. With the exception of 1 patient with uncontrolled disease, all 9 patients were able to taper their steroid dose below the adrenal replacement level during dapsone treatment. Maintenance-phase patients taking 15 mg/d or more of prednisone (n = 5) experienced a mean +/- SEM drop of 67% +/- 7.1% in prednisone dose by 4 months of maximal dapsone treatment and an 84% +/- 3.5% drop in prednisone dose after 8 months of dapsone treatment. CONCLUSIONS: These retrospective study findings suggest that dapsone reduces steroid dependence in patients with pemphigus vulgaris, provided they are in the maintenance phase of their disease. These data support the need for a prospective, randomized trial to confirm these findings.

Adult↗
PubMed2005

Combination antimalarials in the treatment of cutaneous dermatomyositis: a retrospective study.

OBJECTIVE: To observe whether the use of antimalarials in combination resulted in significant improvement in the cutaneous signs and symptoms of patients with dermatomyositis who did not otherwise respond to the use of single-agent antimalarial therapy. DESIGN: Retrospective case series of 17 patients treated between January 1, 1991, and December 31, 2002. SETTING: An ambulatory medical dermatology clinic in an academic center.Patients Patients had adult-onset dermatomyositis with predominantly cutaneous symptoms and a follow-up period at our clinic of at least 6 months. Cases in which it was not possible to assess the effect of treatment on cutaneous symptoms were not included. Intervention Treatment regimens varied and included the use of antimalarials, prednisone, methotrexate, and other medications. MAIN OUTCOME MEASURES: Physician-observed and patient-reported improvement based on erythema, pruritus, and extent of affected skin. RESULTS: Seven of 17 patients experienced at least near clearance in cutaneous symptoms with the use of antimalarial therapy alone: 4 of these patients required combination therapy (hydroxychloroquine sulfate-quinacrine hydrochloride or chloroquine phosphate-quinacrine), while 3 of them responded well to antimalarial monotherapy. The median time required to reach the response milestones on the final working therapeutic regimen was 3 months (mean, 4.8 months; range, 2-14 months). Six patients did not respond significantly to any type of therapy, including nonantimalarials. CONCLUSION: Our experience suggests that a significant subgroup of patients whose skin lesions have been unresponsive to a single antimalarial benefit from combination therapy with hydroxychloroquine and quinacrine or chloroquine and quinacrine, but controlled clinical trials are warranted to assess the extent of benefit.

Adult↗
PubMed2005

Ultraviolet-B recruits mannose-binding lectin into skin from non-cutaneous sources.

Mannose-binding lectin (MBL) is an integral part of the innate immune system and functions as an opsonin by binding to pathogens and certain apoptotic cells to promote their uptake by phagocytes. We recently identified an association of low-producing MBL polymorphisms with adult dermatomyositis (DM). Our model is that MBL deficiency leads to a defect in the clearance of apoptotic debris in the skin, thereby predisposing to photosensitive autoimmune disease. In this study, we sought to determine whether MBL binds within the epidermis, and to determine its source, and potential function of this binding. We demonstrated that the MBL is present in irradiated, but not in non-irradiated skin, and in irradiated skin it is bound to apoptotic keratinocytes (KC). We found that MBL is not made by KC, showing indirectly that it comes from an exogenous source, despite the fact that other complement components are made by KC and upregulated by ultraviolet irradiation. Finally, we demonstrated that non-KC-derived MBL bound to apoptotic KC in vitro and increased the uptake of these cells by dendritic cells. We hypothesize that MBL may facilitate non-inflammatory clearance of apoptotic debris in patients with photosensitive forms of DM.

Adult↗
PubMed2005

The CLASI (Cutaneous Lupus Erythematosus Disease Area and Severity Index): an outcome instrument for cutaneous lupus erythematosus.

We developed and validated a measurement instrument (CLASI-Cutaneous Lupus Erythematosus Disease Area and Severity Index) for lupus erythematosus that could be used in clinical trials. The instrument has separate scores for damage and activity. A group of seven American Dermato-Rheumatologists and the "American College of Rheumatology Response Criteria Committee on SLE (systemic lupus erythematosus)" assessed content validity. After a preliminary session, we conducted standardized interviews with the raters and made slight changes to the instrument. The final instrument was evaluated by five dermatologists and six residents who scored nine patients to estimate inter- and intra-rater reliability in two sessions. Consultation with experts has established content validity of the instrument. Reliability studies demonstrated an intra-class correlation coefficient (ICC) for inter-rater reliability of 0.86 for the activity score (95% confidence interval (CI) = 0.73-0.99) and of 0.92 for the damage score (95% CI = 0.85-1.00). The Spearman's rho (Sp) for intra-rater reliability for the activity score was 0.96 (95% CI = 0.89 to 1.00) and for the damage score Sp was 0.99 (95% CI = 0.97-1.00). Clinical responsiveness needs to be evaluated in a prospective clinical trial, which is ongoing.

Humans↗
PubMed2004

Activation of autoimmunity following use of immunostimulatory herbal supplements.

BACKGROUND: Evidence for the scientific basis of purported therapeutic effects and adverse effects of herbal supplements continues to grow. Many herbal supplements are touted for their immunostimulatory properties, and both in vitro and in vivo experiments have supported this claim. Although this explains their beneficial effects in preventing or curtailing disease, to our knowledge, no immunostimulatory herbal supplements have been reported to exacerbate disorders of immune system overactivity. OBSERVATIONS: We describe 3 patients whose autoimmune disease onset and/or flares correlated with ingestion of herbal supplements with proven immunostimulatory effects. Echinacea and the alga Spirulina platensis are implicated in 2 patients' flares of pemphigus vulgaris, and a supplement containing the algae Spirulina platensis and Aphanizomenon flos-aquae was ingested by a third patient days before both onset and a severe flare of dermatomyositis. The third patient showed heterozygosity for a tumor necrosis factor alpha (TNF-alpha) promoter polymorphism (-308A), leading to increased production of TNF-alpha, which may have predisposed her to developing dermatomyositis. CONCLUSIONS: Immunostimulatory herbal supplements may exacerbate preexisting autoimmune disease or precipitate autoimmune disease in persons genetically predisposed to such disorders. Increased production of TNF-alpha may play a role, although more research is needed to clarify the mechanisms of such phenomena.

Autoimmune Diseases↗
PubMed2004

Photosensitivity in rheumatic diseases.

There have been a number of recent advances in the genetic understanding of photosensitive rheumatic diseases, especially subacute cutaneous lupus erythematosus and dermatomyositis. These advances support the concept that increased numbers of ultraviolet light-induced apoptotic cells in skin lead to a supra-threshold concentration of antigenic peptides. The current genetic data suggest that increased keratinocyte apopotosis can result from increased amounts of TNF-alpha that induce apoptosis due to a ultraviolet light-sensitive TNF promoter polymorphism or to decreased clearance of apototic cells due to polymorphisms associated with decreased serum levels of collectins such as C1q and mannose-binding lectin. These diseases are frequently oligogenic, and other yet to be elucidated genes will, in individual patients, lead to increased numbers of apoptotic cells associated with these cutaneous autoimmune diseases. In the presence of specific MHC class I and II genes, antigen-presenting cells initiate a primary immune response that leads to cutaneous, and likely systemic, autoimmune disease.

Apoptosis↗
PubMed2004

Lupus erythematosus induced by medications, ultraviolet radiation, and other exogenous agents: a review, with special focus on the development of subacute cutaneous lupus erythematosus in a genetically predisposed individual.

Exogenous agents implicated in or suspected of precipitating subacute cutaneous lupus erythematosus (SCLE) and lupus erythematosus (LE) are reviewed. An illustrative case of environmentally induced SCLE is presented. A previously healthy 30-year male homozygous for the tumor necrosis factor-alpha (TNF-alpha) 308. A promoter allele developed SCLE after spending several hours removing fertilizer- and pesticide-containing hay from an agricultural barn in the springtime. The cutaneous eruption soon resolved, only to reappear 3 weeks later on the day the patient re-entered the barn. An environmental agent present in the barn, coupled with springtime ultraviolet light, likely triggered the disease in this immunogenetically susceptible individual.

Adult↗
PubMed2003

Darier's disease associated with an underlying neoplasm in combination with a nodular fibroproliferative disease.

Darier's disease is an autosomal dominant disease that typically arises during the first or second decades of life. It involves the abnormal keratinization of the epidermis, mucosa, and nails. We describe a 74-year-old man with metastatic carcinoma in whom fibroproliferative disease and an acquired variant of Darier's disease developed, both of which may have been paraneoplastic processes.

Adenocarcinoma↗