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Erythematosus
Kinds of Erythematosus Selected AbstractsPulsed dye laser: what's new in non-vascular lesions?JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 7 2007S Karsai Abstract Background and objective, In dermatology, the pulsed dye laser (PDL) is the therapeutic instrument of choice for treating most superficial cutaneous vascular lesions. In addition, clinical experience over the last decade allowed us to treat patients with an ever increasing number of non-vascular indications. The purpose of this report is to summarize and critically appraise the scientific evidence that support the role of PDL in treating non-vascular skin lesions. Methods, A literature-based study has been conducted, including the review of publications over the period January 1995 to December 2006, using the Medline Database. We also included our own experience in managing non-vascular lesions with the PDL. Four sets of preoperative and postoperative photos are presented. Results, For viral skin lesions, PDL proved to be an alternative to other therapy options. This applies particularly to periungual warts and mollusca contagiosa. The mechanism of PDL with inflammatory dermatoses has not yet been elucidated. The effect seems to be better if there is a vascular component to the disease. With most of these indications (such as psoriasis and acne), PDL currently plays a rather minor or complementary role. Regarding collagen remodelling (hypertrophic scars, keloids, stretch marks, and skin rejuvenation), the question of whether a therapy makes sense or not has to be decided from case to case. Conclusion, With PDL, it is possible to achieve good results with numerous, partly less well-known indications (i.e. lupus erythematosu). With other diseases, PDL has so far been considered to be a complementary therapy method or to be in an experimental state. [source] New concepts in antimalarial use and mode of action in dermatologyDERMATOLOGIC THERAPY, Issue 4 2007Sunil Kalia ABSTRACT: Although chloroquine, hydroxychloroquine and quinacrine were originally developed for the treatment of malaria, these medications have been used to treat skin disease for over 50 years. Recent clinical data have confirmed the usefulness of these medications for the treatment of lupus erythematosus. Current research has further enhanced our understanding of the pharmacologic mechanisms of action of these drugs involving inhibition of endosomal toll-like receptor (TLR) signaling limiting B cell and dendritic cell activation. With this understanding, the use of these medications in dermatology is broadening. This article highlights the different antimalarials used within dermatology through their pharmacologic properties and mechanism of action, as well as indicating their clinical uses. In addition, contraindications, adverse effects, and possible drug interactions of antimalarials are reviewed. [source] Fulminant thrombotic thrombocytopenic purpura in two patients with systemiclupus erythematosus and phospholipid autoantibodiesEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 6 2000M. Osman Musa No abstract is available for this article. [source] Osteopontin and the skin: multiple emerging roles in cutaneous biology and pathologyEXPERIMENTAL DERMATOLOGY, Issue 9 2009Franziska Buback Abstract:, Osteopontin (OPN) is a glycoprotein expressed by various tissues and cells. The existence of variant forms of OPN as a secreted (sOPN) and intracellular (iOPN) protein and its modification through post-translational modification and proteolytic cleavage explain its broad range of functions. There is increasing knowledge which receptors OPN isoforms can bind to and which signaling pathways are activated to mediate different OPN functions. sOPN interacts with integrins and CD44, mediates cell adhesion, migration and tumor invasion, and has T helper 1 (Th1) cytokine functions and anti-apoptotic effects. iOPN has been described to regulate macrophage migration and interferon-, secretion in plasmacytoid dendritic cells. Both sOPN and iOPN, through complex functions for different dendritic cell subsets, participate in the regulation of Th cell lineages, among them Th17 cells. For skin disease, OPN from immune cells and tumor cells is of pathophysiological relevance. OPN is secreted in autoimmune diseases such as lupus erythematosus, and influences inflammation of immediate and delayed type allergies and granuloma formation. We describe that OPN is overexpressed in psoriasis and propose a model to study OPN function in psoriatic inflammation. Through cytokine functions, OPN supports immune responses against Mycobacteria and viruses such as herpes simplex virus. OPN is also implicated in skin tumor progression. Overexpression of OPN influences invasion and metastasis of melanoma and squamous cell carcinoma cells, and OPN expression in melanoma is a possible prognostic marker. As OPN protein preparations and anti-OPN antibodies may be available in the near future, in-depth knowledge of OPN functions may open new therapeutic approaches for skin diseases. [source] Pimecrolimus in dermatology: atopic dermatitis and beyondINTERNATIONAL JOURNAL OF CLINICAL PRACTICE, Issue 8 2005Paolo Gisondi Summary Pimecrolimus is a calcineurin inhibitor developed for the topical therapy of inflammatory skin diseases, particularly atopic dermatitis (AD). Pimecrolimus selectively targets T cells and mast cells. Pimecrolimus inhibits T-cell proliferation, as well as production and release of interleukin-2 (IL-2), IL-4, interferon-, and tumour necrosis factor-,. Moreover, pimecrolimus inhibits mast cell degranulation. In contrast to tacrolimus, pimecrolimus has no effects on the differentiation, maturation and functions of dendritic cells. In contrast to corticosteroids, pimecrolimus does not affect endothelial cells and fibroblasts and does not induce skin atrophy. Given the low capacity of pimecrolimus to permeate through the skin, it has a very low risk of systemic exposure and subsequent systemic side-effects. In different randomised controlled trials, topical pimecrolimus as cream 1% (Elidel®) has been shown to be effective, well tolerated and safe in both adults and children with mild to moderate AD. In addition, pimecrolimus has been successfully used in inflammatory skin diseases other than AD, including seborrheic dermatitis, intertriginous psoriasis, lichen planus and cutaneous lupus erythematosus. [source] Childhood discoid lupus erythematosusINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 9 2009Reena Rai MD No abstract is available for this article. [source] Leflunomide in subacute cutaneous lupus erythematosus , two sides of a coinINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 1 2008Anke Suess MD Background, Subacute cutaneous lupus erythematosus (SCLE), a distinct clinical subset of lupus erythematosus, remains a therapeutic challenge, especially in cases resistant to topical and standard systemic therapy. Leflunomide, a novel antirheumatic drug, has shown efficacy in the treatment of systemic lupus erythematosus in pilot studies. Methods, We report two patients with SCLE who demonstrated the spectrum of possible clinical responses to leflunomide therapy. Results, One patient experienced a complete clinical remission of symptoms, whereas the other developed a massive skin reaction which was distinctly related to the commencement of leflunomide therapy. Conclusion, To our knowledge, this is the first time that remission and deterioration of SCLE by leflunomide therapy have been described. [source] Lupus erythematosus associated with erythema multiforme: Rowell's syndromeINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 2007Nadia H. Shadid MD About 45 years ago, the association of lupus erythematosus with erythema multiforme and specific immunological laboratory changes was described for the first time. Since then, several patients with similar constellations of clinical symptoms and laboratory parameters have been reported. Today, this disease is known as Rowell's syndrome. However, the fact that not all patients described previously fit all clinical and immunological characteristics that had been originally reported raised the question if Rowell's syndrome really is an own entity or if the simultaneous occurrence of these clinical and immunological signs is rather incidental. Here, we describe a patient fulfilling most of the criteria of Rowell's syndrome and discuss what is currently known about this rare condition. [source] Discoid lupus erythematosus recurs after plastic surgeryINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 11 2004Bansal Ramesh MD No abstract is available for this article. [source] An unusual association of pemphigus vulgaris with hyperprolactinemiaINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 10 2002MNAMS, Sujay Khandpur MD A 21-year-old unmarried woman presented with oral ulcerations and generalized, itchy, fluid-filled, skin lesions of 10 days' duration. The lesions ruptured spontaneously, resulting in extensive denuded areas covered by crusts. One month prior to this, she experienced pain and enlargement of both breasts with galactorrhea. Her menstrual cycles were normal initially, but later she developed menstrual irregularities. No past history suggestive of any other systemic or skin disease, including atopy or drug allergies, could be obtained. Her family history was not contributory. Dermatologic examination revealed multiple, flaccid bullae and extensive denuded areas of skin covered with crusts over the scalp, face, trunk, and upper and lower limbs (Fig. 1). Bulla spread sign and Nikolsky's sign were positive. The oral mucosa, including the lips, buccal surface, tongue, and palate, showed multiple erosions covered with necrotic slough. The rest of the mucocutaneous and systemic examination was within normal limits. Figure 1. Extensive erosions and flaccid bullae over the trunk with breast enlargement The patient's diagnostic work-up revealed: hemoglobin, 11.2 g%; total leukocyte count, 7400/mm3; differential leukocyte count, P62L34E2M2; erythrocyte sedimentation rate, 34 mm/h. A peripheral blood smear examination, urinalysis, blood sugar, and renal and liver function tests were normal. Venereal Disease Research Laboratory (VDRL) test and enzyme-linked immunoabsorbent assay (ELISA) for human immunodeficiency virus (HIV) were nonreactive. Antinuclear antibody, lupus erythematosus (LE) cell, rheumatoid factor, and anti-dsDNA levels were normal. Serum protein electrophoresis demonstrated increased levels of immunoglobulin G (IgG) antibody. The serum prolactin level was significantly raised to 139.49 ng/mL (normal, 3.6,18.9 ng/mL). The sex hormone levels, however, including follicle-stimulating hormone (FSH), luteinizing hormone (LH), estradiol, and progesterone, were within normal limits. The thyroid hormone profile was also unaltered. Chest X-ray was normal. Ultrasound of the abdomen and pelvis revealed no visceral abnormality and computerized tomography (CT) scan of the pituitary sella showed no adenoma. Mammography was negative for breast malignancy. A Tzanck smear prepared from the base of the erosion showed multiple acantholytic cells and lymphocytes. Histologic examination from an intact vesicle was suggestive of pemphigus vulgaris (PV), showing a suprabasal cleft with acantholytic cells and the basal layer demonstrating a "row of tombstones" appearance (Fig. 2). Direct immunofluorescence (DIF) revealed the intercellular deposition of IgG and C3 throughout the epidermis in a "fishnet pattern." Indirect immunofluorescence (IIF) test performed on rat esophagus for circulating IgG antibody was positive in a titer of 1 : 120. Figure 2. Photomicrograph showing suprabasal cleft with "row of tombstones" appearance, suggestive of pemphigus vulgaris (hematoxylin and eosin, × 40) Based on the clinical and immunohistological features, a diagnosis of PV with idiopathic hyperprolactinemia was made. The patient was treated with bromocriptine mesylate (Tablet Proctinal, Glaxo Wellcome Ltd, India) at a dose of 2.5 mg twice a day. After 2 months of therapy, significant improvement in the skin lesions was observed. The existing lesions re-epithelialized with a drastic reduction in the number and distribution of new vesicles. However, no change in the mucosal erosions was noticed. IIF test demonstrated a lower antibody titer (1 : 40). The breast complaints also improved with a reduction in serum prolactin level to 6.5 ng/mL. The patient refused further treatment as she experienced nausea and dizziness with bromocriptine. After 2 weeks, the disease relapsed with the appearance of new vesicles over the forearms, abdomen, back, and thighs. She again complained of breast tenderness and galactorrhea, and the serum prolactin level was 95 ng/mL. The IgG titer increased to 1 : 120. Hence, treatment with oral prednisolone (2 mg/kg/day) and bromocriptine (2.5 mg twice a day) with an antiemetic was initiated. After 6 weeks, the skin lesions had cleared completely, the breast symptoms had improved, menses had become regular, and the prolactin level had decreased to 4 ng/mL. IIF test was negative for circulating antibody. Steroids were tapered off and maintenance therapy with bromocriptine at a dose of 2.5 mg/day was continued. [source] Facets of lupus erythematosus: panniculitis responding to thalidomideJOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT, Issue 3 2008Simone Wienert Summary Lupus erythematosus profundus or lupus panniculitis is a rare clinical variant of lupus erythematosus, which involves the deep dermis and subcutaneous fat. Diagnosis may be difficult in cases with isolated involvement. Further manifestations of lupus erythematosus may thus be essential for diagnosis, which depends on the clinical picture, histopathology and a positive lesional lupus band test. We report a severe, mutilating case of lupus panniculitis, which responded well to thalidomide. [source] Subacute cutaneous lupus erythematosus with bullae associated with porphyria cutanea tardaJOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT, Issue 3 2007Wiebke K. Peitsch Summary A 58-year-old patient presented with both annular and polycyclic as well as vesicular lesions. Histology revealed an interface dermatitis with focal hyperkeratosis and subepidermal blistering. Antinuclear antibodies were elevated (1 : 1280) and autoantibodies against Ro-SS-A were found. Based on these findings we made a diagnosis of subacute cutaneous lupus erythematosus (SCLE) with blister formation. Additionally, we diagnosed porphyria cutanea tarda (PCT) triggered by alcohol abuse. Treatment with systemic corti-costeroids and low-dose hydroxy-chloroquine led to rapid resolution of the skin changes. SCLE with blister formation is a rare cause of bullous skin eruptions and has to be distinguished from bullous autoimmune diseases as well as from bullous SLE. Recognition of concomitant PCT, which may be associated with all forms of LE, is especially important because of the therapeutic implications, since a reduced dosage of antimalarials is required. [source] Differentiating thrombotic microangiopathies induced by severe hypertension from anemia and thrombocytopenia seen in thrombotic thrombocytopenia purpura,JOURNAL OF CLINICAL APHERESIS, Issue 3 2004J.A. Egan Abstract Thrombotic microangiopathy (TMA) is a recognized complication of malignant hypertension (HTN). Such patients have blood pressures ,200/140 mmHg but the condition is defined by the presence of papilledema and is frequently complicated by acute renal failure. Here we report two patients with severe HTN (systolic ,180 mmHg or diastolic ,120 mmHg), TMA, thrombocytopenia, renal failure, and, in one case, neurological changes (4 of 5 manifestations of the TTP pentad). A 50-year-old male with HTN presented with blurred vision, dizziness, headache, confusion, renal failure, and a TMA (PLT = 39 × 109/L and LD = 2,781 normal <600 U/L). On presentation, BP was 214/133 mmHg and an ophthalmic exam demonstrated no papilledema. With HTN control over 7 days, his platelet count rebounded (220 × 109/L), LD declined (1,730 U/L), and mental status improved. A 60-year-old female with diabetes, HTN, Lupus erythematosus, mild chronic anemia, and thrombocytopenia presented with abdominal pain, shortness of breath, renal failure, and a TMA (PLT = 83 × 109/L and LD = 2,929 U/L). Blood pressures were 180,210/89,111 mmHg and ophthalmic exam demonstrated no papilledema. With HTN control over 8 days, her platelet count rebounded (147 × 109/L), and LD declined (1,624 U/L). Although in both cases a diagnosis of TTP was considered because of overlap with the classic diagnostic pentad, neither received plasmapheresis. TTP is a diagnosis of exclusion, where there is no other likely diagnosis to explain the TMA. In cases of severe HTN (with or without papilledema), the diagnosis of TTP should be held in abeyance until the effect of HTN control can be assessed. J. Clin. Apheresis 19:125,129, 2004. © 2004 Wiley-Liss, Inc. [source] Lupus erythematosus: clinical and histopathological study of oral manifestations and immunohistochemical profile of epithelial maturationJOURNAL OF CUTANEOUS PATHOLOGY, Issue 10 2006Silvia Vanessa Lourenço Background:, Lupus erythematosus (LE) is an autoimmune disease of unknown cause. Prevalence of oral involvement in patients with LE is uncertain but may vary from 9 to 45% in patients with systemic disease and from 3 to 20% in patients with chronic cutaneous involvement. Methods:, Incidence of oral lesions of LE and their clinical aspects were investigated. Their histopathologic features were analyzed, and the status of epithelial maturation was assessed through the expression patterns of cytokeratins. Results:, Twenty-six patients (from 188 examined) presented oral lesions of LE. Most of them were females (19) with systemic disease (11). Clinical aspects of these lesions varied, and lips and buccal mucosa were most affected. Histologically, lesions revealed lichenoid mucositis with perivascular infiltrate and thickening of basement. Cytokeratins profile showed hyperproliferative epithelium, with expression of CK5/6, and CK14 on all epithelial layers, CK16 on all suprabasal layers and CK10 on prickle cell layers only. Conclusions:, Oral lesions of LE show a variety of aspects, and their microscopic features are of a lichenoid mucositis with deep inflammatory infiltrate. Cytokeratins expression patterns are of hyperproliferative epithelium, and this phenomenon must be analyzed in relation to the inflammatory cytokines for a better understanding of the mechanisms of the disease. [source] Herpetic Folliculitis is Usually a Consequence of Varicella Zoster Virus InfectionJOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2005J Blair Skin biopsies of 8 patients diagnosed with herpetic folliculitis by light microscopy were retrieved from the files of the UCSF Dermatopathology Service. Clinical and microscopical features were reviewed and tabulated, and PCR analysis was employed to seek DNA sequences specific for herpes simplex virus (HSV) and varicella zoster virus (VZV). The study group included 4 women and 4 men, ages 15 to 54. Five patients (62%) were immunosuppressed, with underlying conditions including HIV infection, leukemia, rheumatoid arthritis, and lupus erythematosus with polyarteritis nodosa. Microscopically, herpetic cytopathic changes involved the isthmus in 7/8 cases (87%), and involved the sebaceous apparatus in 4/8 cases (50%). Herpetic viropathic changes were not found within eccrine epithelium. A moderate to dense perifollicular infiltrate, comprised mostly of lymphocytes, was evident in 7/8 cases (87%). After PCR expansion of genetic material extracted from the original paraffin blocks, VZV-specific DNA sequences were detected in 8/8 cases. We conclude that herpetic folliculitis is a consequence of VZV infection. Because follicular herpetic infection is often accompanied by a dense perifollicular lymphoid infiltrate, the microscopical presentation can simulate inflammatory skin diseases such as lupus erythematosus. Level sections may be required for a specific diagnosis to be made. [source] Fatal Subcutaneous Panniculitis-Like T-Cell Lymphoma (Sptcl) with Interface Change and Dermal Mucin, A Dead-Ringer for Lupus ErythematosusJOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2005L. Ma We report a 48-year-old male who presented with ulcerated plaques and nodules of the lower extremities. Skin biopsies revealed a dense lymphocytic infiltrate involving the dermis and the subcutis in a lobular and septal pattern. No overt cytological atypia was present. Notably, several features resembling lupus erythematosus were present, including vacuolar interface change and abundant dermal mucin deposition. The patient developed pulmonary nodules, and a lung biopsy showed a perivascular and interstitial lymphoid infiltrate without overt atypia. The cutaneous and pulmonary lymphoid infiltrates showed similar immunohistochemical profiles: CD3+CD4,CD8+/,CD56+. Monoclonal rearrangements of T-cell receptor gamma gene with similar migration patterns were identified from both locations. The patient developed fatal hemophagocytic syndrome, involving liver, spleen, lymph nodes, and bone marrow. This case is amongst rare reports of subcutaneous panniculitis-like T-cell Lymphoma (SPTCL) with systemic involvement. [source] Subcutaneous Panniculitis-Like T Cell Lymphoma Developing in a Patient with Chronic B-Cell Lympocytic LeukemiaJOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2005L Shahabi Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is an unusual peripheral lymphoma most typically presenting with a cytotoxic (CD8-positive, TIA-1-positive) immunophenotype. SPTCLs may have an indolent or highly aggressive clinical course. Histologically, SPTCL may be notoriously difficult to diagnosis. Cases of SPTCL with a deceptively benign appearance similar to that of subcutaneous lupus erythematosus have been described. SPTCL associated with a concomitant systemic leukemia/lymphoma has not been documented in the literature. We report a case of SPTCL arising in a 65-year-old female with a well-established history of B-cell lymphocytic leukemia (BCLL). She presented with two months of recurrent fever and painless erythematous nodules on bilateral lower extremities that were clinically felt to be erythema nodosum. Initial biopsies demonstrated a polymorphous lobular infiltrate with neutrophils, karyorrhexis and lipomembranous change. An excisional biopsy demonstrated an atypical lymphoid population that expressed CD8 and TIA1. PCR analysis confirmed T-cell receptor gene arrangement. The patient was treated with systemic chemotherapy with resolution of her symptoms and complete remission. This is the first well documented case of SPTCL occurring in a patient with long standing B-CLL, and highlights the difficulty of establishing an unequivocal diagnosis of SPTCL. [source] Atypical lymphocytic lobular panniculitisJOURNAL OF CUTANEOUS PATHOLOGY, Issue 4 2004Cynthia M. Magro Background:, Although subcutaneous T-cell lymphoma (SCTCL) is considered an aggressive form of lymphoma, some patients manifest a long waxing and waning phase unaccompanied by constitutional symptoms. Methods:, Twelve patients were prospectively encountered, presenting with a lymphocytic panniculitis accompanied by lymphoid atypia, although not fulfilling criteria for SCTCL. Clinical, histologic, phenotypic, and genotypic analyses were conducted. Results:, There were five men, one boy, and six women; none had symptoms compatible with lupus erythematosus or aggressive SCTCL. All but two had a waxing and waning course of years. Four patients had periodic cytopenias accompanied by fevers. While responding somewhat to prednisone, the lesions relapsed. In one patient, treatment with alemtuzumab (CAMPATH-1) led to complete lesional resolution with no recurrence. Light microscopy showed expansion of the interstices of the fat lobule by mildly atypical lymphocytes of the CD4 subset in 10 biopsies from eight patients; in the other four patients, there was an increase in CD8 lymphocytes. There was diminished expression of CD5 and/or CD7 in the majority of biopsies. Ten of 13 biopsies showed clonal T-cell receptor-, rearrangements. Conclusions:, We apply the term atypical lymphocytic lobular panniculitis to this distinctive form of lymphocytic panniculitis manifesting this light microscopic, phenotypic, and genotypic profile. [source] Scarring alopecia and the dermatopathologistJOURNAL OF CUTANEOUS PATHOLOGY, Issue 7 2001Leonard C. Sperling Background: The evaluation of patients with cicatricial alopecia is particularly challenging, and dermatopathologists receive little training in the interpretation of scalp biopsy specimens. Accurate interpretation of specimens from patients with hair disease requires both qualitative (morphology of follicles, inflammation, fibrosis, etc.) and quantitative (size, number, follicular phase) information. Much of this data can only be obtained from transverse sections. In most cases, good clinical/pathologic correlation is required, and so clinicians should be expected to provide demographic information as well as a brief description of the pattern of hair loss and a clinical differential diagnosis. Results: The criteria used to classify the various forms of cicatricial alopecia are relatively imprecise, and so classification is controversial and in a state of evolution. There are five fairly distinctive forms of cicatricial alopecia: 1) chronic, cutaneous lupus erythematosus (discoid LE); 2) lichen planopilaris; 3) dissecting cellulitis (perifolliculitis abscedens et suffodiens); 4) acne keloidalis; and 5) central, centrifugal scarring alopecia (follicular degeneration syndrome, folliculitis decalvans, pseudopelade). Not all patients with cicatricial alopecia can be confidently assigned to one of these five entities, and "cicatricial alopecia, unclassified" would be an appropriate label for such cases. Conclusion: The histologic features of five forms of cicatricial alopecia are reviewed. Dermatopathologists can utilize a "checklist" to catalog the diagnostic features of scalp biopsy specimens. In many, but not all, cases the information thus acquired will "match" the clinical and histologic characteristics of a form of cicatricial alopecia. However, because of histologic and clinical overlap between the forms of cicatricial alopecia, a definitive diagnosis cannot always be rendered. [source] The cutaneous pathology of lupus erythematosus: a reviewJOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2001A. Neil Crowson The presentation of lupus erythematosus (LE) ranges from a skin rash unaccompanied by extracutaneous stigmata to a rapidly progressive lethal multiorgan disease. The diagnosis and subclassification is traditionally based on the correlation of serological and clinical findings. The latter include a photoinduced skin rash, arthralgia, arthritis, fever, Raynaud's phenomenon, anemia, leukopenia, serositis, nephritis and central nervous sysdtem disease. The conventional classification scheme includes systemic, subacute cutaneous and discoid LE. Recent advances in our understanding of the cutaneous histopathology which correlates with the traditional forms of LE, along with certain novel LE subtypes, are the focus of this review. In addition to the main subtypes of LE, we will discuss associated vasculopathic lesions and the contribution of immunofluorescence microscopy to the diagnosis of LE and related connective tissue disease syndromes. Consideration will be given to unusual variants of LE such as anti-Ro/SSA-positive systemic lupus erythematosus (SLE), bullous SLE, lymphomatoid LE, lupus erythematosus profundus, drug induced LE, linear cutaneous LE, chiblains LE and parvovirus B19-associated LE. [source] Oral manifestations of systemic and cutaneous lupus erythematosus in a Venezuelan populationJOURNAL OF ORAL PATHOLOGY & MEDICINE, Issue 9 2007Jeaneth López-Labady Background:, The aim of this study was to characterize oral lesions in patients with systemic and cutaneous lupus erythematosus (LE) in a Venezuelan group. Methods:, Ninety patients with LE were studied. Oral biopsies were taken from patients who showed oral mucosal involvement. Tissue samples were investigated with histology and direct immunofluorescence techniques for the presence of immunoglobulins G, M, A and complement factor C3. Results:, In 90 patients with LE, 10 patients showed oral lesions related to the disease. Sixteen lesions were investigated. Oral ulcerations accompanied by white irradiating striae occurred in five patients, erythema was observed in five patients and a white homogeneous plaque in one patient. Fifteen lesions demonstrated vacuolar basal degeneration and 12 thickening of the basement membrane histologically. Direct immunofluorescence was negative in three samples. Conclusions:, These findings corroborated that ulcers are not the only manifestation of LE in the oral mucosa. Clinical and histological examinations are significant as immunoproteins are not always found on the oral sample. [source] The use of topical calcineurin inhibitors in lupus erythematosus: an overviewJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 1 2008U Wollina Abstract Lupus erythematosus (LE) shows a broad range of cutaneous symptoms, including acute, subacute and chronic lesions. The gold standard of established topical treatment consists of medium- to high-potency corticosteroids. Because face and neck are often involved, adverse effects of prolonged corticosteroid use are not uncommon. There is a need of steroid-free topical treatment in LE. With the development of topical calcineurin inhibitors, tacrolimus and pimecrolimus, there is an alternative available. The present study reviews the literature data on topical tacrolimus and pimecrolimus for malar rash, subacute lesions and discoid chronic lesions among others. The present data argue for an efficacy of these compounds in acute and subacute cutaneous LE manifestations with a rapid response and only minor side-effects when used as an adjunct to systemic treatment. In chronic discoid LE, hypertrophic plaques do not well respond because of limited penetration. The primary target seems to be the decrease or blocking of cytokine production by activated T lymphocytes. [source] Linear lupus erythematosus following the lines of BlaschkoJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 8 2006M Sàbat [source] Hyperkeratotic nail discoid lupus erythematosus evolving towards systemic lupus erythematosus: therapeutic difficultiesJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 6 2004B Richert ABSTRACT Nail changes occur in about 25% of systemic lupus erythematosus (SLE) cases. Onycholysis has been reported as the most frequent abnormality in SLE. Nailbed hyperkeratosis may be observed in both SLE and discoid lupus erythematosus (DLE). Involvement of the nail apparatus in DLE is extremely uncommon and never restricted to it. We report on a patient in whom the clinical features on the proximal nailfold were similar to those observed on the skin of a patient with typical DLE. This has, to the best of our knowledge, not yet been reported. The patient also exhibited a very distinctive prominent subungual hyperkeratosis. Interestingly, the patient developed biological alterations suggesting a systematization of the disease. Only a combination of systemic corticoids, retinoids and antimalarials was able to achieve nail improvement and this partial resistance to therapy may be explained by the very unusual subungual hyperkeratosis. [source] Abstracts of the 8th Meeting of the Italian Peripheral Nerve Study Group: 62JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2003C Briani Thalidomide seems to be effective in the treatment of cutaneous forms of lupus erythematosus refractory to other therapies. Peripheral neuropathy is the most severe side effect, but the incidence of neuropathy and its relation to thalidomide doses are still unclear. We prospectively monitored 12 patients treated with thalidomide for cutaneous lupus erythematosus in order to estimate the occurrence of side effects, particularly peripheral neuropathy. A total of 12 female patients, median age 38,6 years (range 26,56), with subacute or chronic cutaneous lupus erythematosus were considered. The patients were treated with low dose thalidomide (starting dose 100 mg, tapered to 50 mg/day or 50 mg alternative day) for up to 18 months. The average follow-up period was 8,6 months (range 2,18). Prior to, and regularly during treatment patients underwent neurological evaluation and electrophysiological study of at least 8 nerves in the 4 arms (ulnar, median, sural, peroneal nerves). At recruitment, one patient presented a sensory-motor peripheral neuropathy. Of the remaining 11 patients, six did not present electrophysiological evidence of neuropathy, one had a carpal tunnel syndrome and four showed slowing of ulnar nerve velocity at elbow. No patients developed neuropathy neither worsening of electrophysiological parameters during thalidomide treatment. The most common side effect was tremor, always reversible after withdrawing or reducing thalidomide. Paresthesias, somnolence, amenhorrea, constipation were also present. Only one patient had to stop the therapy for the occurrence, 10 days after taking 50 mg of thalidomide, of a severe, stabbing, "zoster-like" thoracic pain, which disappeared upon withdrawal of the drug. Started again on thalidomide, the symptoms reappeared and the patient definitely interrupted the therapy with benefit. All the 11 patients who continued on the therapy presented a significant improvement or remission of the cutaneous alterations. These preliminary data seem to indicate that low dose thalidomide is efficacious and tolerable for cutaneous lupus erythematosus. Peripheral neuropathy seems not to be a major side effect. A longer follow-up and the study of more patients are needed to confirm the results. [source] Neutrophilic Figurate Erythema of InfancyPEDIATRIC DERMATOLOGY, Issue 2 2008ANNALISA PATRIZI M.D. Neutrophilic figurate erythema of infancy is a rare inflammatory dermatosis which is part of the figurate inflammatory dermatoses of infancy and is considered a variant of annular erythema of infancy. The disease is clinically characterized by annular erythematous lesions, sometimes with a polycyclic configuration, and histologically by a dermal neutrophilic infiltration with leukocytoclasia. Differential diagnosis mainly includes clear-cut severe diseases with a well-known etiology, such as neonatal lupus erythematosus and its variant erythema gyratum atrophicans transiens neonatale, erythema chronicum migrans and erythema marginatum rheumaticum, and diseases of unknown origin and with less clear limits, such as erythema annulare centrifugum and its variant familial annular erythema. Anamnesis, laboratory findings, clinical features, and histology allow the correct diagnosis and therefore, having excluded severe diseases, parents should be reassured as neutrophilic figurate erythema of infancy, as the annular erythema of infancy, is a benign disease, in most cases unassociated with other conditions and usually self-limiting over a few months, even though a chronic course may rarely occur. Our case was characterized by a chronic persistent course and by a complete resolution of the lesions only during febrile episodes. [source] Analysis of Compact Fluorescent Lights for Use by Patients with Photosensitive ConditionsPHOTOCHEMISTRY & PHOTOBIOLOGY, Issue 4 2009Rachel S. Klein Ultraviolet radiation (UVR) is hazardous to patients with photosensitive skin disorders, such as lupus erythematosus, xeroderma pigmentosum and skin cancer. As such, these patients are advised to minimize their exposure to UVR. Classically, this is accomplished through careful avoidance of sun exposure and artificial tanning booths. Indoor light bulbs, however, are generally not considered to pose significant UVR hazard. We sought to test this notion by measuring the UV emissions of 19 different compact fluorescent light bulbs. The ability to induce skin damage was assessed with the CIE erythema action spectrum, ANSI S(,) generalized UV hazard spectrum and the CIE photocarcinogenesis action spectrum. The results indicate that there is a great deal of variation amongst different bulbs, even within the same class. Although the irradiance of any given bulb is low, the possible daily exposure time is rather lengthy. This results in potential daily UVR doses ranging from 0.1 to 625 mJ cm,2, including a daily UVB (290,320 nm) dose of 0.01 to 15 mJ cm,2. Because patients are exposed continually over long time frames, this could lead to significant cumulative damage. It would therefore be prudent for patients to use bulbs with the lowest UV irradiance. [source] Narrow-band UVB-induced Externalization of Selected Nuclear Antigens in Keratinocytes: Implications for Lupus Erythematosus Pathogenesis,PHOTOCHEMISTRY & PHOTOBIOLOGY, Issue 1 2009Adam Reich The aim of this study was to analyze whether sera obtained from patients with lupus erythematosus (LE) react with membrane structures found on keratinocytes irradiated with narrow-band ultraviolet B (NB-UVB). We applied atomic force microscopy (AFM) to visualize cell surface structures expressing nuclear antigens upon apoptosis following NB-UVB irradiation. Immortalized human keratinocytes (HaCaT) were cultured under standard conditions, irradiated with 800 mJ cm,2 NB-UVB light and imaged by AFM mounted on an inverted optical microscope. It was observed that NB-UVB irradiation provoked significant alterations of the keratinocyte morphology and led to the membrane expression of antigens recognized by anti-La and anti-Ro 60 kDa sera but not by antidouble-strand DNA sera. The presence of La and Ro 60 kDa antigens on keratinocyte surfaces after NB-UVB irradiation was limited mainly to the small bleb-like protrusions found on the keratinocytes by AFM. A closer investigation by AFM also revealed that some structures positively stained with anti-Ro 60 kDa serum were also located submembranously. We hypothesize that the externalization of some nuclear antigens because of NB-UVB exposure might be responsible for exacerbation of skin symptoms in patients suffering from LE. [source] Photosensitivity in lupus erythematosusPHOTODERMATOLOGY, PHOTOIMMUNOLOGY & PHOTOMEDICINE, Issue 5 2004Noah Scheinfeld Background: Lupus erythematosus is a systemic disease process that may manifest with a variety of internal and cutaneous findings. Photosensitivity is one the most common manifestations of lupus erythematosus. In patients with lupus erythematosus, there is a relationship between exposure to ultraviolet light, autoantibodies, genetics and other factors in the development of photosensitivity. Methods: Literature was reviewed on the topics of lupus erythematosus and photosensitivity discussed together and separately. The suggested mechanisms for their relationship were reviewed and analyzed. Results: Photosensitivity's relationship to and influence on the systemic manifestations of lupus remain to be defined. Mechanisms for photosensitivity might include: modulation of autoantibody location, cytotoxic effects, apoptosis induction with autoantigens in apoptotic blebs, upregulation of adhesion molecules and cytokines, induction of nitric oxide sythase expression and ultraviolet-generated antigenic DNA. Tumor necrosis factor , also seems to play a role in the development of photosensitivity. Conclusion: The basis for photosensitivity in lupus has yet to be fully defined. It is more commonly associated with subacute and tumid lupus erythematosus than with other variants. Anti-Ro antibodies appear to relate to photosensitivity. Tumor necrosis factor , polymorphisms appear to be important in some variants of lupus with photosensitivity. There is no sin que non antibody or mutation of photosensitivity in lupus. In patients with lupus, more work needs to be done to define the mechanisms of photosensitivity. [source] Thalidomide inhibits UVB-induced mouse keratinocyte apoptosis by both TNF-,-dependent and TNF-,-independent pathwaysPHOTODERMATOLOGY, PHOTOIMMUNOLOGY & PHOTOMEDICINE, Issue 6 2003Kurt Q. Lu Background: Thalidomide is an anti-inflammatory pharmacologic agent that has been utilized as a therapy for a number of dermatologic diseases. Its anti-inflammatory properties have been attributed to its ability to antagonize tumor necrosis factor-alfa (TNF-,) production by monocytes. However, its mechanism of action in the skin is not known. Purpose: To test our hypothesis that thalidomide may antagonize TNF-, production in the skin, we used a mouse model for acute ultraviolet-B (UVB) exposure, a known stimulus for inducing this cytokine. Results: A single bolus dose of thalidomide (either 100 or 400 mg/kg) given immediately before UVB exposure (40,120 mJ/cm2) inhibited, in a dose-dependent manner, sunburn cell formation (i.e. keratinocyte (KC) apoptosis as defined by histologic appearance and confirmed by terminal transferase mediated biotinylated dUTP nick end labelling staining) in mouse skin biopsy specimens. However, this agent did not affect the formation of cyclobutane pyrimidine dimers, a measure of UVB-induced DNA damage, which is an early event associated with apoptosis. RNase protection assays confirmed that high (400 mg/kg), but not low (100 mg/kg), doses of thalidomide inhibited the UVB-induced increase in steady-state TNF-, mRNA. Additionally, our in vitro data using neonatal mouse KCs showed that thalidomide prevented UVB-induced cell death (JAM assay). The antiapoptotic effects of thalidomide can be reversed by the addition of exogenous recombinant mouse TNF-, and hence reconstituting UVB-induced programmed cell death. The inhibition of sunburn cell formation by low-dose thalidomide in the absence of TNF-, inhibition suggests that other, unidentified mechanisms of apoptosis inhibition are active. Conclusions: These data suggest that the anti-inflammatory effects of thalidomide can affect UVB injury, and may, in part, explain its action in photosensitivity diseases such as cutaneous lupus erythematosus. [source] | ||||||||||