RDS | Rheumatologic Dermatology Society



2013 Annual Meeting AbstractsRheumatologic Dermatology SocietyOctober 26, 2013San Diego, CaliforniaLUPUSDrug-Induced Subacute Cutaneous Lupus Erythematosus Isolated to the Hands Due to Topical Terbinafine Cream.Adnan Mir, Jonathan Leventhal, Sarika Ramachandran, Rishi Patel, David E. Cohen and Andrew G. Franks, Jr.The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, NYBackground: Drug-induced subacute cutaneous lupus erythematosus (SCLE) is the most common form of drug-induced lupus erythematosus (DILE). ?It is comparable to idiopathic SCLE in terms of clinical, histologic and serologic features. ?Anti-Ro/SS-A is as prevalent in both drug-induced and idiopathic SCLE. ?Drugs associated with SCLE include calcium channel blockers, angiotensin-converting enzyme inhibitors, thiazide diuretics, terbinafine and tumor necrosis factor (TNF)-alpha antagonists.Methods: We describe the case of a 34-year-old female foot masseuse who presented with an eruption isolated to the dorsal hands and interdigital spaces for 2 months while she was using topical terbinafine on her clients. ?Ironically, the patient’s general physician treated this eruption empirically with oral terbinafine for 16 days for a presumed diagnosis of dermatophytosis. ?The patient did not improve but reported the development of a transient generalized urticarial reaction in other parts of her body. Results: Physical examination revealed scaly, erythematous plaques on the interdigital and dorsal surfaces of the fingers. There were no lesions on the head or neck or other locations. The patient’s ANA was positive at a titer of 1:40, in a speckled pattern, and SS-A antibody was positive at 1.4 (nl < 1.0). Histopathology was consistent with subacute cutaneous lupus erythematosus. Conclusion: ?We propose that our patient developed localized SCLE due to topical terbinafine cream from chronic topical exposure as a foot masseuse. In addition, when inadvertently challenged with oral administration of terbinafine, she developed an urticarial reaction consistent with systemic allergic sensitization. ?We believe that further investigation of the pharmacodynamics of topical terbinafine and its potential to induce subacute cutaneous lupus erythematosus is warranted.Prognostic significance of anti-nuclear antibody titers in discoid lupus patients Daniel Grabell, BA, MBA1, Christy Wang, MD1, Roselynn Nguyen1, MD, Jack O’Brien, BA1, Beverley Adams-Huet, MS2 , Benjamin F. Chong, MD, MSCS1 1 Department of Dermatology, 2 Department of Clinical Sciences University of Texas Southwestern Medical Center, Dallas, TX Background: Anti-nuclear antibodies (ANAs) have been associated with generalized involvement and systemic symptoms in discoid lupus erythematosus (DLE) patients, suggesting that ANA may be a risk factor for systemic lupus erythematosus (SLE) development. However, these studies did not longitudinally follow DLE patients with and without positive ANAs to assess ANA’s prognostic value. Objective: To determine ANA’s clinical significance at DLE diagnosis, we compared disease courses of DLE-only patients (DLE patients without SLE) with and without positive ANAs respectively, for at least two years. Methods: A retrospective cohort study of 26 DLE-only patients seen at University of Texas Southwestern Medical Center and Parkland Memorial Hospital between May 1987 and June 2013 was performed after local institutional review board approval. All were diagnosed with DLE based on clinicopathological correlation and had ≥ two annual follow-ups. Patients who did not have ANA drawn within the first year of DLE diagnosis, had concomitant SLE at DLE diagnosis, or had a history of other autoimmune diseases were excluded. Indirect immunofluorescence tests using Hep2 cells were used to measure ANA titers, with positive titers being ≥1:160. Clinical data at initial visit (t=0) and subsequent annual follow-ups (t=one, two years, etc.) were collected for up to six years. The number of body parts defined by Cutaneous Lupus Activity and Severity Index (CLASI) was the primary outcome. Secondary outcomes included number of SLE criteria met and oral medications. Results: 26 of 46 DLE-only patients passed initial screening. Nine had positive ANA titers (ANA+), including one who developed SLE four years after DLE diagnosis. 17 had negative ANA titers (ANA-) initially, including two who became ANA+ at year 3 and 5, respectively. No DLE ANA- patient developed SLE. DLE ANA+ patients had increased CLASI body parts, fulfilled significantly more SLE criteria (even excluding positive ANA titers), and required higher numbers and potency of medications at diagnosis than DLE ANA- patients. Over time, DLE ANA- patients had significantly higher rates of increase in number of CLASI body parts (p=0.03), SLE criteria (p<0.0001), oral medications (p=0.0007), and potency of medications (p=0.005) than DLE ANA+ patients Conclusions: Although DLE ANA+ patients had worse baseline disease activity, over time, DLE ANA- patients exhibited greater disease spread. Our findings may result from efficacy of aggressive treatments in ANA+ patients or differences in disease evolution between the groups. Larger prospective studies comparing disease courses in DLE ANA+ and ANA- patients using CLASI scores and other disease activity markers will be conducted.MISCELLANEOUS CONNECTIVE TISSUE DISEASEFacial erythromelalgia: A rare entity to consider in the differential diagnosis of connective tissue diseases1Mital Patel MD, 1Alisa N. Femia MD, 2A. Brooke Eastham MD, 1Janice Lin MD, 1Ruth Ann Vleugels MD, MPH1Department of Dermatology, Brigham and Women’s Hospital and Harvard Medical School, Boston, MA; 2Harvard Combined Dermatology Residency Program, Boston, MA ?We present an interesting case of a 53-year-old male with four years of an erythematous facial rash associated with significant burning and exacerbated by sun exposure. Given his predominant facial involvement, he was initially diagnosed with rosacea and actinic damage. After a lack of clinical response, he was then diagnosed with connective tissue disease based on a biopsy that revealed vacuolar interface alteration with prominent perivascular and periadnexal lymphoid infiltration and mucin deposition. Treatment with both systemic corticosteroids and hydroxychloroquine provided minimal clinical response. He was subsequently referred to our connective tissue disease clinic for additional therapy. On exam, the patient had prominent erythema and edema of both the face and ears. Additional history, specifically relief with cooling measures, established a clinical diagnosis of erythromelalgia. After initiating therapy with aspirin, pentoxifylline, and gabapentin the patient experienced significant improvement of his condition. Erythromelalgia is a rare condition characterized by episodic erythema, swelling, warmth, and burning pain which primarily occurs on the extremities, though may rarely manifest on the ears and face. Exposure to heat may trigger these episodes, and patients frequently report that cooling alleviates their symptoms. This disorder can lead to significant long-term morbidity; therefore timely recognition and initiation of therapy is essential. Given there is no confirmatory or diagnostic test, the patient's history and physical examination during the episodes establishes the diagnosis. Patients often receive numerous incorrect diagnoses prior to being recognized as having erythromelalgia, and those with facial involvement can be misdiagnosed as having connective tissue disease. Although biopsy findings are often non-specific in erythromelalgia, histopathologic findings of connective tissue disease have been reported. This case illustrates the difficulty in diagnosing atypical cases of erythromelalgia. Specifically, it highlights that patients with facial involvement can be misdiagnosed as having connective tissue disease, particularly when skin biopsy findings support this entity. This is the second reported case of clinically consistent erythromelalgia with biopsy demonstrating vacuolar interface dermatitis and mucin deposition, underscoring the need for physicians who follow patients with connective tissue disease to be aware of the clinical presentation of facial erythromelalgia. Calcinosis Cutis Occurring in Association with Autoimmune Connective Tissue Disease**David A. Wetter, M.D.Objective: To describe characteristics and treatment of patients with calcinosis cutis in the clinical setting of autoimmune connective tissue disease. Design: Retrospective study. Setting: Tertiary referral center (Mayo Clinic, Rochester, Minnesota). Patients: Seventy-eight patients with calcinosis cutis and autoimmune connective tissue disease between 1996 and 2009. Main Outcome Measures: Clinical features, treatments, and outcomes of patients with calcinosis cutis in the clinical setting of autoimmune connective tissue disease. Results: Of 78 patients (mean age at onset of calcinosis cutis, 40.1 years), 64 (82%) were female. The following diseases were associated with calcinosis cutis: dermatomyositis (n=30) with classic (n=15), juvenile (n=14), and amyopathic (n=1) subtypes; systemic sclerosis with limited cutaneous scleroderma (n=24); lupus panniculitis (n=4); systemic lupus erythematosus (n=2); mixed connective tissue disease (n=4); overlap connective tissue disease (n=6); undifferentiated connective tissue disease (n=6); polymyositis (n=1); and rheumatoid arthritis (n=1). Therapy for calcinosis cutis consisted of medical treatment alone (n=19; 24%), surgical therapy alone (n=11; 14%), combined medical and surgical treatment (n=17; 22%), and no treatment (n=30; 38%). Diltiazem was the most commonly used medical therapy, with 9 of 17 patients having a partial response. Twenty-eight patients had surgical excision of 1 or more lesions of calcinosis cutis; 22 had a complete response and 5 had a partial response. Conclusions: Dermatomyositis and systemic sclerosis were the most common autoimmune connective tissue diseases associated with calcinosis cutis. Although no treatment was uniformly effective, surgical excision of symptomatic lesions and medical treatment with a calcium channel blocker (diltiazem) provided benefit for some patients.**From Arch Dermatol. 2012;148(4):455-462 (Balin SJ, Wetter DA, Andersen LK, Davis MDP)DERMATOMYOSITISIdentification of clinical features and risk factors associated with calcinosis in adult patients with dermatomyositisAntonia Valenzuela1, Lorinda Chung1,3, Livia Casciola-Rosen2, Antony Rosen2, David Fiorentino1,31Stanford University School of Medicine, Division of Immunology and Rheumatology2Johns Hopkins University School of Medicine, Department of Rheumatology3Stanford University School of Medicine, Department of DermatologyBackground: Prior studies have estimated that up to 20% of adult dermatomyositis (DM) patients suffer from calcinosis. ?Although calcinosis is related to persistent disease activity, poor treatment adherence, and therapy refractoriness in juvenile DM (JDM) patients, risk factors for calcinosis in the adult DM population have not been extensively studied. ?Antibodies to nuclear matrix protein 2 (NXP-2) have been associated with calcinosis in JDM patients but only one study has shown a trend for this association in adult DM. ?We aimed to determine the prevalence of calcinosis and to identify associated clinical features in our cohort of extensively phenotyped adult DM patients. ?Methods: This is a cross-sectional study of 126 patients diagnosed with DM at Stanford University Medical Center between 01/2006 and 01/2013. Calcinosis was defined as the presence of calcium deposition in the skin and/or subcutaneous tissues as determined by physical examination and/or radiography. Logistic regression was used to obtain odds ratios (OR) relating calcinosis to various clinical features in multivariate analyses. Results: 94% of patients had DM-specific or myositis-specific autoantibodies (against NXP-2, transcription intermediary factor-gamma (TIF-), melanoma differentiation antigen 5 (MDA-5), sumoyl activating enzyme (SAE1/2), Mi-2, or Jo-1). A total of 14 (11%) patients had calcinosis. The association between the autoantibodies to NXP-2 and to TIF- and calcinosis reached statistical significance in separate multivariate analyses adjusting for digital ulcers, disease duration, interstitial lung disease and the autoantibody to Ro52 (OR=15.5, 95%CI 2.01-119.9, p=0.008 and OR= 0.2, 95%CI 0.01-0.99, p=0.04), while MDA-5 was no longer significantly associated with calcinosis. Digital ulcers and disease duration were strongly associated with calcinosis in all multivariate models, independent of the underlying autoantibody present (Table 1). Conclusion: Calcinosis was a relatively uncommon clinical feature in our cohort of DM patients. Our data support an association with NXP-2 autoantibodies as well as digital ulcers. A common vascular mechanism may underlie the development of both calcinosis and digital ulcers in patients with DM.Table 1. Multivariate analysesMultivariate model OR95% CIp-valueNXP-2 analysisDisease duration1.21.03–1.420.018Digital ulcers12.32.24–67.90.003ILD4.70.91–24.20.06Ro524.30.78–23.070.09NXP-215.52.01–119.90.008MDA-5 analysisDisease duration1.21.03–1.370.01Digital ulcers11.51.21–1090.03ILD3.50.67–17.80.13Ro522.20.49– 10.20.30MDA-50.70.06 –9.200.80TIF- analysisDisease duration1.21.02 – 1.390.02Digital ulcers12.22.17 – 69.30.004ILD1.70.34 – 9.110.4Ro523.70.75 – 18.90.10TIF-0.20.01 – 0.990.04ILD= Interstitial lung disease, OR=Odds ratios, CI=Confidence intervalsDermatomyositis Associated with Hematologic Malignancies: Report of Two Cases and DiscussionMaria Aleshin, BA, David Geffen School of Medicine at UCLAScott Worswick, MD, UCLA Department of Medicine, Division of DermatologyDermatomyositis (DM) is an idiopathic inflammatory myopathy which is characterized clinically by symmetric proximal muscle weakness and unique cutaneous findings. While dermatomyositis primarily affects the skin and muscle, it can also have systemic manifestations including arthralgias, dysphagia and interstitial pneumonitis. Furthermore, 20 to 25% of cases of dermatomyositis have been linked to an internal malignancy, most commonly carcinomas of ovarian, pancreatic and colorectal origin. Some cases of dermatomyositis resolve following cancer treatment suggesting a paraneoplastic disease origin, while others follow an independent course suggesting an alternative disease mechanism. To date, few studies have associated dermatomyositis with hematologic malignancies. Here we report two cases of dermatomyositis in patients with myelodysplastic syndrome (MDS) and monoclonal gammopathy of undetermined significance (MGUS) and propose a common immune mediated mechanism possibly contributing to these disease processes. DM is a truly systemic disease with broad implications for a patient’s health. In addition to the classical teaching of the overlap of DM and solid malignancies, the above reports present further evidence for the association between DM and hematological disorders.The Use of Static and Dynamic Diffusion-Weighted Magnetic Resonance Imaging in the Diagnosis and Treatment of Dermatomyositis1Sarika Ramachandran, MD; 1Alisa Femia, MD; 2Eric Sigmund, PhD; 1Andrew Franks, MDNew York University School of Medicine, New York, NY, The Ronald O. Perelman Department of Dermatology1 and the Department of Radiology2Dermatomyositis (DM) is an inflammatory disorder that primarily affects skin and skeletal muscle and can result in destruction of muscle function and structure and impaired quality-of-life. ?Although skin histopathology and muscle enzymes may provide diagnostic assistance, diagnosis is often delayed. In addition, therapeutic benefit and optimal dosing regimens may be difficult to determine due to difficulty distinguishing between muscle disease activity and damage. Therefore, it would be highly desirable to find an objective and non-invasive measure that would improve and expedite diagnosis, help monitor therapeutic benefit, and help distinguish between disease activity and damage. Magnetic resonance imaging (MRI) is a non-invasive tool that may accomplish these objectives, and we propose using static and dynamic diffusion-weighted MRI for this purpose, specifically employing state-of-the-art compressed sensing strategies to accelerate dynamic multiple echo diffusion tensor acquisition technique (MEDITATE) in skeletal muscle. We plan to perform proximal muscle diffusion-weighted MRI (DWI) in forty normal controls and forty DM patients identified through clinical and laboratory data and/or skin biopsy, and to perform structure and vascular-sensitive DWI at rest and during and after physical exertion. We plan to compare this data with clinical markers of DM, and patients with DM will be followed during the course of immunosuppressive treatment with repeated imaging after 6 weeks. ??We hypothesize that the results of this study will show that these novel compressed sensing strategies will increase sensitivity of the MEDITATE technique to detect kinematic changes in muscle, that these imaging techniques will provide further evidence that DM pathology includes reduction in microvascular flow and myofiber disruption and degradation, that dynamic imaging before and after exercise will correlate with analogous measures such as electromyography, and that static and dynamic diffusion MRI can help predict response to immunosuppressive therapy. This data and development of this imaging technique is hoped to aid in earlier detection of DM, to provide further insight into the pathophysiology of DM, and to help monitor disease activity, thereby providing an objective measure to aid in the adjustment of therapeutic regimens.Validation of the Cutaneous Dermatomyositis Disease Area and Severity Index: characterizing severity and assessing responsiveness to clinical changeCynthia O. Anyanwu, 1,2 BS, David Fiorentino,3 MD, PhD, Lorinda Chung,3 MD, Yanli Wang,4 MS, Joyce Okawa,2 RN, Kasey Carr,2 RN, Kathleen Joy Propert,4 ScD, Victoria P. Werth,1,2 MD. 1Philadelphia Veterans Affairs Medical Center, Philadelphia, PA,2Department of Dermatology, University of Pennsylvania School of Medicine, Philadelphia, PA3Department of Dermatology, Stanford University School of Medicine, Stanford, CA4Department of Biostatistics and Epidemiology, University of Pennsylvania, Philadelphia, PABackground/Purpose: Translational research and clinical trials necessitate validated outcome measures to reliably assess disease progression and treatment efficacy. The Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI), a disease-specific and skin-based outcome measure, was developed for use in clinical trials and longitudinal patient assessment. The reliability and validity of this instrument have been established.1 The goal of this study is to assess responsiveness of this tool and characterize severity and clinical response of cutaneous dermatomyositis (DM) using the CDASI.Methods: In this two-center prospective database study the skin of patients with clinical or histologic evidence of DM was evaluated. Patients were evaluated using clinical instruments including the CDASI, a physician global assessment (PGA) 5-point Likert scale measuring disease severity (none, mild, moderate, severe, extremely severe), a PGA 0 – 10 cm visual analog scale (VAS) of disease severity and a PGA 3-point Likert scale that captured change in disease severity since the last visit (improved, worse, no change). The severity analysis compared CDASI scores for patients with mild disease to those with moderate and severe disease according to the PGA 5-point Likert. The PGA 3-point Likert and PGA VAS were used to evaluate responsiveness. Clinical response was defined as a rating of “improved” on the PGA 3-point Likert or a change in VAS scores of at least 2 cm. Statistical analysis included logistic regression models using generalized estimating equations to account for correlation among patients. A receiver operating characteristic curve was used with each model to determine cutoffs.Results: A total of 199 patients from two sites completed up to 12 study visits each. Study participants were 79% female and 78% Caucasian. Disease subtype was classified as classic in 65% of patients and skin predominant in 35%. Separate site-specific analyses were performed due to interactions between study site and score in both the severity and responsiveness analyses. Baseline CDASI scores at the two sites range from 0 to 47 (median 17) and 0 to 48 (median 21). Data collected at one site resulted in a cutoff of 19 to differentiate mild from moderate and severe disease while at the second site CDASI scores of 14 or less characterized mild disease. Compared to the PGA VAS, the PGA 3-point Likert may be a less reliable measure of clinical response. Using a PGA VAS to assess responsiveness we found that improvement in CDASI scores of 4 or 5 indicates a clinically significant change.Conclusion: Inter-rater variations in the use of the external PGA Likert and VAS gold measures may account for the differences between sites. The above results suggest that the CDASI is a valid and responsive tool for the evaluation of cutaneous dermatomyositis but randomized controlled trials are needed to confirm these results.?Reference:Goreshi R, Okawa J, Rose M et al. Evaluation of reliability, validity, and responsiveness of the CDASI and the CAT-BM. J Invest Dermatol2012; 132:1117-24.Management of cutaneous dermatomyositis: results of a stepwise therapeutic strategy Cynthia O. Anyanwu, 1,2 BS, Rui Feng,3 ScD, Kasey Carr,2 RN, Joyce Okawa,2 RN, Victoria P. Werth,1,2 MD1Philadelphia Veterans Affairs Medical Center, Philadelphia, PA 2Department of Dermatology, University of Pennsylvania School of Medicine, Philadelphia, PA3Department of Biostatistics and Epidemiology, University of Pennsylvania, Philadelphia, PABackground: Dermatomyositis requires a multifaceted approach to treatment that considers the involved organs, potential adverse effects of medications, patient preference, and comorbidities. Treatment of dermatomyositis (DM) is conventionally approached in a stepwise manner but the literature is lacking strong evidence to support use of this therapeutic strategy. Methods: Using a prospective, longitudinal cohort study of 102 (DM) patients, we describe the result of a stepwise therapeutic strategy in the management of DM. DM was managed using a stepwise algorithm wherein patients with primarily skin disease initially received antimalarials. Immunosuppressive agents such as mycophenolate, methotrexate and azathioprine were added when antimalarials were not sufficient. Intravenous immunoglobulins were administered when dermatomyositis was refractory to antimalarials and immunosuppressives. Systemic corticosteroids were used with any combination of treatment agents to manage myositis or severe cutaneous disease activity. The Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) was used to assess disease severity at each visit. Results: Of 102 dermatomyositis patients, 30% were managed with antimalarials, 64% required the use of immunosuppressive agents and 7% did not receive systemic therapy during the study period. When patients presented with only skin disease 48% received antimalarials alone, 44% received immunosuppressive or immunomodulatory agents and 8% did not require systemic therapy. Prednisone was included in the treatment regimen for 46% of all patients and 23% of skin-only patients. For 14% of all study patients the current treatment regimen was not sufficient so therapy was escalated at the last study visit. After a median follow-up of 21 months (range 1 - 56) the median final CDASI activity score for all patients was 11 (range 0 - 52) and 13.5 (0 - 52) for patients with skin-only disease. Median duration of treatment for all patients was 21 months (range 1 - 192). In 13% of patients exposed to antimalarials a cutaneous eruption attributable to antimalarial use was noted. Conclusions: These results indicate that antimalarials alone are frequently insufficient for skin-predominant patients and management of DM often requires the use of second-line agents. Patients continue to have mild disease activity despite long periods of therapy. Our data supports the use of the above-mentioned treatment algorithm and highlights the need for clinical trials to determine the efficacy of these treatment regimens.SYSTEMIC SCLEROSISAdult and juvenile systemic sclerosis: two cases illustrating differences in disease presentation and potential treatment options1Alisa N. Femia, MD; 1Janice Lin, MD, MPH; 1Christina Lam, MD; 1Mital Patel, MD; 1 A. Brooke Eastham, MD; 2Fatma Dedeoglu, MD; 1Ruth Ann Vleugels, MD, MPH 1Brigham and Women’s Hospital and Harvard Medical School, Department of Dermatology, Boston, MA; 2Boston Children’s Hospital, Division of Allergy and Immunology, Boston, MASystemic sclerosis is an inflammatory and fibrotic disorder that can affect multiple organs and may lead to significant functional limitation, morbidity and mortality. The disorder presents a unique challenge to physicians, as data regarding therapy is limited, and no treatment has been proven to modify overall disease course. Systemic corticosteroids may be included in therapeutic regimens, especially for patients with severe skin or internal organ involvement. However, use of corticosteroids is controversial due to some data linking them to scleroderma renal crisis, although no causal relationship has been established. Mycophenolate mofetil has emerged as a therapy capable of decreasing modified Rodnan skin scores in patients with diffuse systemic sclerosis, particularly in patients with early-onset disease; however, although this is supported by recent prospective data, no randomized controlled trials have confirmed this benefit. Evidence regarding treatment for juvenile-onset systemic sclerosis is even more limited, partially due to the fact that the disease is exceptionally rare, with an estimated incidence of 0.05 per 100,000 persons. In this presentation, we discuss two cases: a 28 year-old woman who presented with Raynaud’s phenomenon and sclerosis of the digits, face, trunk, and extremities after being admitted with severe cardiac, pulmonary, and renal involvement, and an 8 year-old girl who presented with sclerosis of the digits, trunk, and extremities without substantial internal organ involvement. ?Both patients were managed successfully with a combination of systemic corticosteroids and mycophenolate mofetil. To our knowledge, this constitutes the first reported case of mycophenolate mofetil for the treatment of systemic sclerosis during childhood. We use these cases to help define clinical characteristics of juvenile-onset systemic sclerosis, and to illustrate the clinical differences between juvenile and adult-onset disease. In addition, we discuss the controversy regarding systemic corticosteroids and scleroderma renal crisis, consider the existing data for treating adults with systemic sclerosis with mycophenolate mofetil and emphasize that prolonged treatment with this agent may be necessary prior to appreciating clinical benefit, and propose mycophenolate mofetil as a treatment option for juvenile-onset systemic sclerosis.MORPHEAThe role of skin trauma in the distribution of morphea lesions: a cross sectional survey of the Morphea in Adults and Children (MAC) cohort IV Daniel Grabell, BA, MBA; Clifford Hsieh, BS; Rachel Andrew, MD; Kathryn Martires, MD; Andrew Kim, MD; Rebecca Vasquez, MD; Heidi Jacobe, MD, MSCS Background: Morphea is a sclerosing disorder of the skin and subcutaneous tissue. Distribution of trauma-induced skin lesions have been described using “isotopic” and “isomorphic” terms in chronic GVHD which has features in common with morphea. These distribution patterns have not been well-examined in morphea patients. Objective: To determine whether patients enrolled in the Morphea in Adults and Children (MAC) cohort exhibit preferential distribution of skin lesions in areas of skin trauma in an isotopic or isomorphic distribution. The “isotopic response” is defined as the development of a second, unrelated disease in the same area as previous healed disease or injury whereas the isomorphic response of Koebner is the manifestation of disease in an area predisposed to developing it. In addition patient demographics, clinical characteristics, and impact on quality of life in patients with trauma-induced morphea lesions will be evaluated. Methods: A cross-sectional analysis of the MAC cohort at the University of Texas Southwestern Medical Center in Dallas, Texas. Patients were included from those enrolled in the MAC cohort from June 2007 through March 2012. Of the 329 patients in the cohort, 277 were excluded due to the following: age <4 years (N=9), insufficient data on variables of interest (N=29), indeterminate subtype (N=8), and lack of inciting event or isomorphic distribution of lesions (N=231). Patients were evaluated using modified Rodnan skin scores (MRSS), the Dermatology Life Quality Index (DLQI), and analysis of specific clinical variables using a standardized case report form. Fisher’s exact test was used for comparisons of categorical variables, and unpaired t-test was used for comparisons of continuous variables between groups. P values less than 0.05 were considered significant. Results: 52 (16%) of patients in the MAC cohort had skin trauma associated lesions, 21 (6%) in an isotopic and 31 (9%) in an isomorphic distribution. There was a difference in the MRSS between the groups, with the isotopic mean score of 13.8 and the isomorphic mean score of 5.3 (P = 0.004, 95% CI= 3.08 to 13.92). The DLQI has a mean of 8.4 in isotopic patients versus 4.1 in isomorphic patients (P = 0.009, 95% CI= 1.18 to 7.50). The LoSSI and the LoSDI were 46 and 24 respectively for the isotopic group and 25 and 20 for the isomorphic group. There was no significant difference in MRSS and DLQI between those with skin trauma-induced morphea and the overall MAC cohort. Conclusion: Some patients with morphea likely have a reaction in response to skin trauma resulting in cutaneous sclerosis. This implies that patients with morphea may be at risk for extension or reactivation of their disease in areas of skin trauma and procedures should be undertaken with caution. VASCULITISD-Dimer Levels as a Marker of Disease Activity in Skin Disease:Case Reports of Cutaneous Polyarteritis Nodosa and Recurrent Urticaria with Case SeriesMark G Kirchhof1, Agnes Y Y Lee2, and Jan P Dutz1,31Department of Dermatology and Skin Science, University of British Columbia, Vancouver, BC, Canada; 2Vancouver Coastal Health, Vancouver General Hospital and Associate Professor, Department of Medicine, University of British Columbia, Vancouver, BC, Canada; 3Child and Family Research Institute, University of British Columbia, Vancouver, British Columbia, Canada.Biochemical markers of disease allow clinicians to monitor disease severity, progression and response to treatment. C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) are commonly used biochemical markers of inflammatory disease. D-dimers are small protein fragments generated by fibrinolysis of a thrombus or blood clot. D-dimer assays are often used as aids in the diagnosis venous thrombosis. Damage to blood vessel walls leads to activation of the coagulation cascade, thrombus formation and D-dimer release into the blood stream. ?D-dimer levels may be elevated in forms of vasculitis including Henoch-Schonlein purpura (HSP), Kawasaki disease and Churg-Strauss syndrome. Here we report two cases where disease activity correlated with D-dimer levels. ?The first case is 51 year old female patient with a diagnosis of cutaneous polyarteritis nodosa. ?The second case is a 29 year old male with recurrent urticaria. ?We further present a case series of D-Dimer analysis in a variety of inflammatory conditions including HSP, leukocytoclastic vasculitis, Behcet’s syndrome, lupus, Still’s disease, graft versus host disease (GVHD), urticarial vasculitis and pyoderma gangrenosum (n = 15). ?D-dimer levels were compared with CRP values and clinical assessment of disease activity. ?The sensitivity of the D-dimer test was 0.91 and the specificity was 0.50, while the sensitivity of the CRP test was 0.89 and the specificity was 0.38. ?Our findings suggest that measuring D-dimer levels may be useful as a clinical marker of vasculopathy in autoimmune and autoinflammatory disease. We show here the use of D-dimer measurements as a marker of vasculocentric/vasculopathic inflammation and reveal the possibility that vascular enodothelial damage may be ongoing in many inflammatory conditions.PITYRIAIS RUBRA PILARISPityriasis Rubra Pilaris: Evaluating Patient Quality of Life1A. Brooke Eastham MD, 2Alisa N. Femia MD, 3Lisa K. Pappas-Taffer MD, 3Misha A. Rosenbach MD, 2Ruth Ann Vleugels MD, MPH1Harvard Combined Dermatology Residency Program, Boston, MA; 2Department of Dermatology, Brigham and Women’s Hospital and Harvard Medical School, Boston, MA 3Department of Dermatology, University of Pennsylvania, Philadelphia, PAAlthough pityriasis rubra pilaris (PRP) is often considered a debilitating skin condition that can negatively impact patient quality of life (QoL), no formal studies of PRP and its impact on QoL exist. We aimed to determine the impact of PRP on QoL and to elucidate which independent variables are associated with poor QoL in patients with PRP. An online survey consisting of validated skin-specific QoL instruments, including Skindex-29 and Dermatology Life Quality Index (DLQI), as well as the validated global medical QoL instrument Short Form-36 (SF-36) was administered to members of the online PRP support group. Additionally, we collected information on demographic characteristics and PRP-related sequelae including alopecia, photosensitivity, palmoplantar keratoderma, ectropion, joint pain associated with PRP onset, and hospitalization. A total of 116 patients with dermatologist-diagnosed PRP completed all eligible surveys. Those with active disease (n = 85) were asked to complete all surveys, while those with inactive disease (n = 31) were asked to complete all except the DLQI and Skindex-29. Quality-of-life scores from patients with PRP were compared to pre-existing data from the same QoL measures performed in patients with other dermatologic or non-dermatologic conditions, and the level of statistical significance was set at P = 0.01. According to functioning scores from Skindex-29, patients with active PRP had significantly worse QoL than all other dermatologic conditions to which it was compared (P = < 0.01) including vulvodynia, dermatomyositis, cutaneous lupus erythematosus (CLE), epidermolysis bullosa, eczema, pemphigus vulgaris, psoriasis, acne, and cutaneous T-cell lymphoma, amongst others. With respect to Skindex-29 emotions scores, patients with active PRP demonstrated significantly worse QoL than all of these same skin diseases (P = 0.01), except for vulvodynia (P = 0.011), dermatomyositis (P = 0.193) and CLE (P = 0.033). With respect to Skindex-29 symptoms scores, PRP was found to have significantly worse QoL than all these same skin diseases (P = < 0.01), except for epidermolysis bullosa (P = 0.025). There was significant correlation between the DLQI and Skindex-29 scores (all P values <0.001). With respect to SF-36, those with active PRP have worse QoL in role physical and bodily pain domains than patients with recent myocardial infarction (MI), hypertension, type 2 diabetes mellitus (T2DM), and the general population (all P values < .01). Analysis of vitality, social functioning, and role emotional indicated worse QoL than those with congestive heart failure, CLE, recent MI, hypertension, T2DM, and the general population (all P values < .01). Factors related to poor QoL in patients with PRP include alopecia and joint pain. This study indicates that active PRP profoundly impacts patient QoL. Additional systematic investigation is necessary to further elucidate treatment options for PRP and to determine the ways in which treatment may impact QoL over time. ................
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