Diagnostic investigations

A thorough history and physical examination is required for the correct diagnosis of CSVV. This should include screening tests for infections, connective tissue disease, medication usage, and cancer. Laboratory screening test are always required both to confirm the diagnosis and to determine the Figure 3. Cutaneous small vessel vasculitis. Central necrosis on the distal lower extremity. (Courtesy of Dr. Kelly Barham, Wake Forest University School of Medicine, Winston-Salem, NC, USA.) Figure 3....

References

Abe, J., Kotzin, B.L., Jujo, K., et al. 1992. Selective expansion of T cells expressing T-cell receptor variable regions V beta 2 and V beta 8 in Kawasaki disease. Proc. Natl. Acad. Sci. USA 89, 4066. Asano, T., Ogawa, S. 2000. Expression of monocyte chemoat-tractant proytein-1 in Kawasaki disease the anti-inflammatory effect of gamma globulin therapy. Scand. J. Immunol 51, 98. Brown, T.J., Crawford, S.E., Cornwall, M.L., et al. 2001. CD8T lymphocytes and macrophages infiltrate coronary artery...

Dermatologie features

ACLE consists of transient erythematous lesions, sometimes with a slight edema or a fine scaling, Division of Rheumatology, University of Padova, Via Giusti- Tel. + 49-8212190 fax + 49-8212191. E-mail address riccardo.rondinone unipd.it (R. Rondinone). which are generally localized on the face (localized ACLE), with a peculiar (but not exclusive) involvement of the malar areas and the bridge of the nose, the so-called 'malar' or 'butterfly rash'. Sometimes the rash also involves the extensor...

T Cells and Dendritic Cells in Immuno Mediated Skin Pathology

Karin Loser, Jenny Apelt, Stefan Beissert* Department of Dermatology, University of M nster, M nster, Germany Autoimmune diseases play an increasingly important role in public health systems of the First World since according to the American Autoimmune Related Diseases Association (AARDA) about 20 of the US population is affected by autoimmune symptoms (http www.aarda.org qa_frames.html). Within the most prevalent autoimmune diseases in dermatology are cutaneous as well as systemic lupus...

The Dermatomyositis Skin Severity Index DSSI

The DSSI (Carrol et al., in press) has been closely modelled on the PASI, which to date is the most successful and the dominant outcome instrument for psoriasis. The basis for the practically identical design of the DSSI and the PASI is the clinical observation that the skin lesions of both diseases share characteristics that are measured by the PASI. In exceptional cases the two diseases can present serious differential diagnostic challenges. However, the experience is that psoriasis is only...

Mycophenolate mofetil

Mycophenolate mofetil (MMF) was initially evaluated in RA and several years later also licensed for the treatment of organ transplantation. Because MMF inhibits specifically T- and B-lymphocyte proliferation by interfering with the inosine monophosphate dehydrogenase, it has emerged as an alternative in SLE mainly for patients refractory to other treatments. The growing interest on MMF in SLE is reflected by a supplement of the journal 'Lupus' to this drug (Hughes and D'Cruz, 2005). In recent...

Other laboratory features

Other laboratory abnormalities have been reported in SCLE patients at various rates in different studies (Sontheimer, 1989). Those include elevated erythrocyte sedimentation rates (15-60 ), elevated gamma globulin levels (30-50 ), hypo-complementemia (15-25 ), leukopenia (20-50 ), anemia (5-50 ) thrombocytopenia (0-40 ) rheumatoid factor (35-50 ), circulating immune complexes (40-60 ) and positive LE cell prep (10-75 ). During the early phase of lesions there can be difficulty in distinguishing...

Diseases

Ilaria Cavazzanaa'*, Angela Tincania, Pierfranco Riboldib, Pier Luigi Meronib aServizio di Reumatologia e Immunologia Cl nica, Spedali Civili di Brescia, Cattedra di Immunologia Clinica, University of Brescia, Italy bAllergy, Clinical Immunology & Rheumatology Unit, Department of Internal Medicine, IRCCS Istituto Auxologico Italiano, University of Milan, Italy Systemic autoimmune disorders are characterized by the occurrence of autoantibodies most of them display an association with a...

Polyarteritis Nodosa

PAN affects men and women equally at all ages, with a predominance between 40 and 60 years. Its etiology remains unknown for most of the patients, with HBV-related PAN accounting for less than 10 of all currently diagnosed cases (Guillevin et al., 2004). It is a necrotizing angiitis, whose main manifestations are weight loss fever asthenia cutaneous lesions peripheral neuropathy (mononeuritis multiplex) renal, musculoskeletal and or gastrointestinal tract involvement hypertension and or...

Prevalenceepidemiology

Systemic scleroderma appears worldwide and affects all races. Women overall are affected approximately three times as often as men and even more often during the late childbearing and early menopausal years. Although, no strong racial predilection has been reported, certain incidence data suggest, that blacks have a higher risk of developing the disease than whites. The annual incidence has been estimated to be between 0.6 and 19 cases Evaluation of skin thickness on a scale from 0 (normal) to...

Key points

Cutaneous manifestations of RA include three principal reaction patterns (i) extravascular palisading granulomatous inflammation (ii) interstitial and or subcuticular neutrophilia and (iii) active vasculopathy encompassing lymphocyte-dominant, neutrophil-rich, and granulomatous vasculitis. The most widely recognized skin lesion is the rheumatoid nodule. Rheumatoid nodules correlate with more severe arthritis, higher levels of RF, and with an increased incidence of RV. CV can be classified by...

Separate measurements of disease activity and damage

The differentiation between activity and damage in our scores is unusual for dermatological scores, Contribution of body areas to the total score in different cutaneous outcome instruments Contribution of body areas to the total score in different cutaneous outcome instruments however, this distinction is established for scores of SLE, where these aspects are commonly separated. This separation leads to two separate scores results for each patient. We have chosen to calculate the score for...

Clinical manifestations

The wheals may contain purpuric foci. Patients may also present with livedo reticularis, nodules, and bullae. Patients with the hypocomplementemic form may have extracutaneous manifestations such as fever, malaise, and myalgia as well as lymph-adenopathy, hepatosplenomegaly, GI symptoms (abdominal pain with or without nausea and or diarrhea), respiratory symptoms (laryngeal edema, dyspnea, chronic obstructive pulmonary disease (COPD)), and or ocular involvement...

Histopathology

Palpable purpura and papular lesions such as urticaria, usually correspond to leukocytoclastic or lymphocytic vasculitis of the small vessels of the dermis, while nodules are preferentially associated with vasculitis of arterioles or small vessels at the junction of dermis and the subcutis or in the subcutis. Necrosis and livedo develop when either small or larger vessels or both are involved. The hallmark histopathologic feature of purp-uric lesions is leukocytoclastic vasculitis of the small...

Treatment

Since the severity, activity, and the overall outcome of MC largely vary among patients, the disease behaviour is often unpredictable during the clinical follow-up (Ferri et al., 2002a, 2004). In the majority of cases, MC can affect, directly and or indirectly, the outcome of the patients. The cumulative 10th year survival of MC patients is significantly lower compared to age- and sex-matched general population (Ferri et al., 2004). A fatal outcome is the result of two or more concomitant...

Acute phase reactants

The acute-phase response can be assessed by measuring different phase reactants. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are most frequently used for disease activity measurement (Dawes et al., 1986). Both measures correlate with disease activity, especially joint swelling, but not with pain. ESR is the cheapest, but can be easily influenced by anaemia and hype-rglobulinaemia frequently present. CRP is more sensitive. Persistent elevated acute-phase response is...

Pathophysiological hypothesis

The clinical association of respiratory tract infections with acute episodes of HSP suggests the triggering role of infectious agents. Secretory IgA in the mucosal lining plays a major role in the defence against exogenous antigens. A defect in the specific IgA1 antibody response at the mucosal level similar to the one reported in IgAN (De Fijter et al., 1996) could favour the antigen penetration and stimulate the systemic immune system. In the circulation, the antigens can be captured by...

D

Figure 6. a) Severe chrysiasis marked blue slate grey discoloration affects the entire face, most obviously involving the eyelids and forehead. b) Lateral forehead biopsy (H + E x 50) shows focal aggregates of dark pigment (curved arrows) in the reticular and papillary dermis. c) At high power (H + E 312 x ) perivascular deposits (straight arrows) are seen concentrated around blood vessels in the reticular dermis and around vessels within sweat glands. d) Electron microscopy (x 12,000)...

Antiphospholipid syndrome

Interesting microvascular alterations have been observed in patients affected by the anti-phospholipid syndrome (APS). Studies in anti-phospholipid patients evaluated the relationship between anti cardiolipin antibodies ACL (found in about 40-50 of patients suffering from SLE) and skin microcirculatory changes or vascular symptoms in 51 consecutive SLE patients (Bongard et al., 1995). Twenty-two patients (43.1 ) showed positive ACL (IgG 22 (5-60) GPL IgM 5 (3-16.5) MPL median titre and range)...

Other biological compounds

Cytokines of the IL-12 (IL-12) family including IL-12, IL-23, and IL-27 are known to regulate Th1-cell responses. In addition, it recently turned out that IL-23 via stimulating T-cell IL-17 production plays an important role in autoimmune inflammation (Hunter, 2005). Accordingly, a monoclonal antibody to the human IL-12 p40 subunit (anti-IL-12p40), which is shared with IL-23 has been developed for the treatment of autoimmune diseases. In a first clinical trial, this antibody was evaluated for...

Microvascular involvement

Diffuse endothelial cell dysfunction (ECD) documented by several techniques occurs commonly in adult and childhood systemic vasculitis (Bacon, 2005). Similar ECD is also seen in connective tissue diseases (CTDs). The mechanisms probably relate to inflammatory cytokines such as tumour necrosis factor (TNF). The particular role of vas-culitic, as opposed to synovial or internal organ inflammation, may be release of secondary mediators directly into the blood stream where they can reach distant...

Stefano Bombardieri Chiara Baldini

Rheumatology Unit, Department of Internal Medicine, University of Pisa, Pisa, Italy Sjogren's syndrome is a chronic autoimmune disorder of unknown aetiology characterized by the dysfunction and destruction of exocrine glands (Ramos-Casals et al., 2005). Sjogren's syndrome is one of the most common systemic autoimmune diseases and it may occur alone, as a primary condition (primary Sjogren's syndrome pSS), or in association with other connective tissue diseases, including rheumatoid arthritis...

Anakinra

Anakinra, a recombinant-methionyl human IL-1Ra, has been shown to reduce joint inflammation and swelling, cartilage destruction, and bone resorption in several animal models of RA (Lebsack et al., 1991 Bendele et al., 1999). The only side effects that appeared to be closely linked with administration of anakinra were skin reactions at the injection site (Bendele et al., 1999) (Fig. 7). Such reactions were the most frequent adverse events, and their frequency and severity increased with...

Riccardo Rondinonea Stefania Banob Luca Iaccarinoa Andrea Doriaa

ADivision of Rheumatology, University of Padova, Padova, Italy bDivision of Dermatology, University of Padova, Padova, Italy Lupus erythematosus (LE) is a multisystemic autoimmune disease that can occur with a wide spectrum of clinical manifestations. The skin is frequently involved, either as an isolated finding, i.e. when no other sign or symptom of systemic involvement can be disclosed, or as a part of a complex syndrome with multiple organ involvement. Based on dermatologic and pathological...

Heteropolymer ETI104

A heteropolymer (HP) system was developed to allow efficient removal of pathogens from the bloodstream through an immune adherence like mechanism (Lindorfer et al., 2001). Immune adherence is a physiological mechanism for the removal of immune complexes. When a foreign substance, an antigen, enters the bloodstream, the immune system develops antibodies that bind to it specifically. The resulting antigen-antibody complex activates the complement system resulting in the formation of the...

AntiB cell therapy

Specific autoantibodies are the serological hallmark of SLE. Therefore, it is quite logic to address B lymphocytes, the precursors of immunoglobulin-producing plasma cells, in therapeutic intervention. In systemic autoimmune diseases, B cells are thought to play a major role, and animal models indicate that such diseases are blocked in mice deficient in B lymphocytes. Furthermore, B cells may also be important in e.g. presenting autoantigens and promoting the breakdown of peripheral T-cell...

Livedo reticularis

LR is a mottled red or bluish discoloration of the skin with a netlike pattern. It is caused by stagnation of blood in dilated superficial capillaries and venules. Livedo may occur in normal subjects on cold exposure. The term cutis marmorata is given to this physiological livedo, which is very common, especially in children. It consists of a symmetrical, regular mottling localized mainly on the limbs. LR is a skin manifestation of a large variety of pathological states.Different clinical...

Pathogenesis

Various autoantibodies against nuclear (antinuclear antibodies) and cytoplasmic antigens are found in up to 20 of the patients with all the inflammatory myopathies (Dalakas, 1990, 1991, 1995, 1997, 2001c Engel and Emslie-Smith, 1989 Karpati and Carpenter, 1993 Engel et al., 1994 Dalakas and Karpati, 2001). The antibodies to cytoplasmic antigens are directed against ribonuc-leoproteins that are involved in translation and protein synthesis and include various synthetases and translation factors....

List of Contributors

Department of Dermatology, University of Pennsylvania, USA Department of Dermatology, University of M nster, M nster, Germany Division of Rheumatology, University of Cape Town Health Sciences Centre, Cape Town, and the Rosebank Clinic, Johannesburg, South Africa. Rheumatology Unit, University Hospital L, Sacco, Via GB Grassi 74, 20157 Milan, Italy Rheumatology Unit, Department of Internal Medicine, University of Pisa, via Roma 67, 56126 Pisa, Italy Division of Dermatology, University of Padova,...

Differential diagnosis

Differential diagnosis of oral and mucocutaneous disease Complex aphthosis HSV infection Inflammatory Bowel disease Reiter's syndrome Erythema multiforme Lichen planus 7.2. Differential diagnosis of oral genital and ocular mucocutaneous disease Mucous membrane pemphigoid. The overlap of inflammatory bowel disease, such as Crohn's disease and chronic ulcerative colitis and BS is well recognized, particularly when extra co-lonic manifestations of inflammatory bowel disease such as aphthosis,...

The validation process

The design of an instrument is often a reflection of biometric necessities as well as of clinical judgement and knowledge. The design process of an instrument can be creative, but the validation process is well established and needs to be documented in detail. Many instruments used in dermatology are poorly validated. This lack of validation may mean that the results of studies may be hard to interpret and that it is unclear if Attributes that should be assessed for a new measurement scale...

Angiogenesis

Bushy Capillary

A large range of different morphologic features of capillary neoformation may be observed in patients with the secondary RP (Maricq et al., 1983). Highly tortuous and arborized capillary loop clusters, often surrounded by dropout of normal capillary loops are a characteristic feature of angiogenesis (Fig. 4). The main morphologic hallmark of angiogenesis is the clustering of tortuous capillaries with a pronounced shape heterogeneity, including thin or large meandering and bushy capillaries...

Annegret Kuhn

Department of Dermatology, University of D sseldorf, D sseldorf, Germany The clinical expression of skin involvement in lupus erythematosus (LE) is very common and extremely heterogeneous. Therefore, it has been difficult to develop a unifying concept of the various cutaneous manifestations of LE, and much attention has been paid to the issue of classifying this disease from the dermatological perspective in the past (Sontheimer, 1997 Kuhn et al., 2004). In 1977, Gilliam (1977) initially...

Genital ulcerations

Other Uses Pethargy Test

Genital ulcers are one of the cardinal signs of BS with a frequency of about 85 . They are oval or round with punched-out appearence. They usually begin as a papule, pustule or necrotic crust that ulcerate within a short period (Fig. 2). Genital ulcers of BS can be painful. Their borders are regular and oedematous and their base are covered with a yellow fibrin. We have recently formally studied the fate of these genital ulcers in a sizeable group of patients (Mat et al., in print). If they are...

CTL4Ig

Complete activation of T cells requires a CD28-mediated costimulatory signal. This signal is inhibited by abatacept, a fully human, recombinant, soluble fusion protein, which binds to B7-1 and B7-2 on antigen-presenting cells. This prevents downstream effects on B cells, macrophages, and synoviocytes. Abatacept was already used in some thousand patients exhibiting a good safety profile. In RA, phase II and III trials show an efficacy in clinical signs and symptoms and also in preventing...

Secondary forms of HSP

Henoch-Schonlein purpura nephritis (HSPN) has been described in association with hypersensitivity. Indeed, several drugs such as ciprofloxacin, acetyl-salicylic acid, vancomycin, carbidopa levodopa, cocaine, ACE inhibitors, carbamazepine and strep-tokinase have been implicated in HSP induction (Disdier et al., 1992 Moots et al., 1992 Kaneko et al., 1993 Drago et al., 1994 Chevalier et al., 1995 Niedermaier and Briner, 1997 Prajapati and Cas-son, 1997 Sola et al., 1997 Michail et al., 1998)....

Deoxyspergualin

Deoxyspergualin (DSG) is a synthetic derivate of spergualin, originally isolated from Bacillus later-osporus, and licensed for reverse graft rejection in some countries. It is proven to be effective in animal models, such as experimental autoimmune encephalomyelitis and murine lupus. The precise mode of action is not known, but in vitro-studies indicate blocking of cell cycle progression in effector T cells following antigen stimulation and inhibition in the production of interferon gamma....

Histological characteristics

Images Vasculitis The Bowels

Severe RV results from a systemic arteritis of small to medium arteries. Histological-ly, this is a leukocytoclastic vasculitis with endothelial swelling, polymorphonuclear leukocyte invasion of vessel walls with necrosis, leukocytoclasis, and extravasation of red blood cells. Peripheral neuropathy presenting as mononeuritis, mononeuritis multiplex, or distal symmetric neuropathy may be the first sign. Neuropathy is assumed to result from vasculitis of the vasa nervorum. Gangrene of the digits,...

Panniculitis

Cutaneous eruptions are comprised of recurrent crops of erythematous, edematous and tender subcutaneous nodules, usually 1-2 cm in diameter, or sometimes larger. In lobular panniculitis, lesions are usually symmetrically distributed and occur most often on the thighs and lower legs. They usually regress spontaneously, but are replaced by hypopigmented and atrophic scars due to fat necrosis. Occasionally, they may become sup-purative. In septal panniculitis, nodular lesions are located primarily...

Diagnosis

Localization and features of cutaneous lesions associated with joints and gastro-intestinal symptoms are very suggestive of HSP diagnosis. However, several other forms of leucocytoclastic vasculitis such as acute infantile haemorragic oedema (AIHE), hypersensitivity vasculitis, lupus erythe-matosus (LE), Wegener's disease and microscopic polyangiitis (MPA) can have a similar clinical presentation. None of the circulating IgA abnormalities described above are specific or constant. If specific...

Outcome Measures in Cutaneous Autoimmune Disease Dermatomyositis and Lupus Erythematosus

Department of Dermatology, University of Pennsylvania, Philadelphia, PA, USA 1. Introduction to outcome measures in cutaneous autoimmune disease The ability to measure, to describe, to classify and to diagnose diseases is the prerequisite for successful clinical research. Diagnosis and classification of cutaneous autoimmune diseases have made enormous progress during the latter half of the last century. In general, we now have a solid frame of reference for clinical research even though some of...

Microscopic polyangiitis

MPA, previously considered the microscopic form of PAN, is now defined as a systemic necrotizing vasculitis that clinically and histologically affects small-sized vessels (i.e. capillaries, venules or art-erioles) without granulomata. MPA is associated with segmental necrotizing glomerulonephritis and anti-MPO ANCA. In practice, MPA is difficult to distinguish from PAN (see above, Section 4.3) and in most early populations studied, MPA was not identified as a separate entity. Hence, skin...

The Skin as an Immunologic Organ

Giampiero Girolomonia, Gianpaolo Tessaria, Jan D. Bosb aDepartment of Biomedical and Surgical Sciences, Section of Dermatology, University of Verona, bDepartment of Dermatology, Academic Medical Center University of Amsterdam, The Netherlands 1. Introduction the skin as an organ of defence Human skin is the largest organ of the body that provides a protective barrier to ensure that exogenous 'noxious' agents do not affect the homeostasis of the organism. Being situated at the interface between...

Agents targeting Tcells

In a preliminary clinical trial, an antibody binding to CD2 (siplizumab), which was expressed on memory effector T-lymphocytes, was successfully evaluated in psoriasis (Bayes et al., 2003). An antibody directed against CD3 (visilizumab), a component of the T-cell receptor complex, which is expressed on all T-lymphocytes, is currently investigated for its efficacy in graft-versus-host disease and previously has been reported to have some therapeutic activity in psoriasis but may not be further...

Main clinical cutaneous manifestations

Polyarteritis Nodosa

The spectrum of clinical vasculitis-related lesions is wide and includes erythema, purpura, papules, pustules, nodules, livedo, necrosis, ulcerations and or bullae. These different lesions are often associated, giving rise to a pleomorphic clinical picture, that is not specific to any of the systemic vasculitides, granulomatous or otherwise. Palpable purpura and petechiae are unquestionably the most frequent manifestations (Figs. 1 and 2). Lesions usually begin as tiny red macules that later...

Local corticosteroids

Initial treatment usually includes daily application of a formulation containing a medium strength topical corticosteroid (e.g., triamcinolone ace-tonide 0.1 ). If this does not provide adequate relief, a more potent topical corticosteroid such as clobetasol propionate 0.05 , betamethasone dipropionate 0.05 , diflorasone diacetate 0.05 , or amcinonide 0.1 can be tried. Daily application of these products to lesional skin for 2 weeks followed by a 2-week rest period of treatment can lessen the...

Diagnostic investigations and differential diagnoses

When cutaneous signs are associated with general symptoms and or other organ or system involvement of vasculitis, differential diagnoses are primarily other systemic diseases, like systemic lupus erythematosus, and secondary vasculitides, attributed to infections, neoplasia, hematologic malignancies or drugs. Indeed, many drugs have been reported to be potential causative agents of vasculitis (Table 5). Differential diagnoses of isolated purpuric lesions are numerous and relatively easy to make...

Histologic features

Non-inflammatory thrombosis in small arteries and or veins throughout the dermis and the subcutaneous fat tissue are the main histological findings Figure 7. Diffuse anetoderma in SLE-related APS. Figure 7. Diffuse anetoderma in SLE-related APS. (Alegre and Winkelmann, 1988 Smith et al., 1990). Endarteritis obliterans, characterized by a narrowing of the vascular lumen with endothelial cell proliferation and fibro-hyalinization of the vessel wall, is sometimes associated with or may even be the...

Dermatologie aspects of Antiphospholipid Antibody Syndrome

Camille Francesa'*, Jean-Charles Piettea, Ronald A. Ashersonb'c aDepartment of Dermatology, Allergology, 4, rue de la Chine, Paris 75020, France bDepartment of Autoimmune Diseases, Institut Clinic de Medicina i Dermatologia, Hospital, Clinic, Barcelona, Catalonia, Spain cDivision of Rheumatology, University of Cape Town Health Sciences Centre, Cape Town, and the Rosebank The first definition of the antiphospholipid antibody syndrome (APS) described recurrent arterial and venous thrombosis and...

Richard D Sontheimer

Department of Dermatology, University of Oklahoma Health Sciences Center, Oklahoma City, OK, USA 1. Introduction and historical perspective In the 1960-1970s, the study of human lupus ery-thematosus (LE) was undergoing a revolution resulting from the description and characterization of various autoantibody-autoantigen systems as disease markers (e.g., Ro SS-A, La SS-B, U1RNP, Sm).1 That such autoantibody systems were found to be associated with specific clinical features of LE and associated...

Detection of antiphospholipid antibodies aPL in dermatology

Theoretically, all the skin manifestations secondary to a non-inflammatory thrombosis whatever the size of cutaneous involved vessels should lead clinicians to look for aPL as for other causes of thrombophilic states. The presence of classical risk factors for thrombosis such as pregnancy, post-partum, smoking, or use of estrogen-containing oral contraceptive pill should not exclude this research as these classical risk factors can act as co-factors for thrombosis in APS (Asherson et al.,...

Bullous skin lesions in lupus erythematosus

A variety of primarily blistering diseases have been reported to occur in patients with SLE, in particular, dermatitis herpetiformis (Davies et al., 1976) bullous pemphigoid (Miller et al., 1978), pemphigus erythematosus (Chorzelski et al., 1968), pemphigus foliaceus (Blanchet et al., 1981), epidermolysis bullosa acquisita (Dotson et al., 1981), and linear IgA dermatoses (Lau et al., 1991). In addition, further types of vesicular and bullous skin lesions can be associated with different...

Acute cutaneous lupus erythematosus

Lesions Face Aids

Acute cutaneous lupus erythematosus (ACLE) usually occurs in association with systemic manifestations preceding by weeks or months the onset of a multisystem disease (Watanabe and Tsuchida, 1995 Wysenbeek et al., 1992 Yung and Oakley, 2000). Sun exposure is a common exogenous factor to be capable of precipitating ACLE (Kuhn et al., 2001a Wysenbeek et al., 1989), and some patients even report an exacerbation of their systemic symptoms after exposure to sunlight. Furthermore, infections,...

Gangrene

Gangrene resulting from arterial occlusion may develop in all vasculitides involving medium-sized or large arteries. Initially, gangrene is characterized by a blue-black color of the extremities, with sharply demarcated borders. The main differential diagnosis is arterial thrombosis associated with atherosclerosis and or emboli. Angiography can only visualize occlusion or stenosis of arteries and is usually not helpful in distinguishing between these different pathogenic processes. The presence...

Conclusion and outlook

The enormous progress in biotechnology as well as in the improved understanding of the underlying pathomechanisms of several autoimmune diseases has paved the road for the development of novel, more specific, and hopefully more efficient therapeutics strategies. In many cases, biologics have already given the physician novel, highly potent, and often patient-friendly drugs at hand in additional to the already existing armamentarium for autoimmune disorders. At present, the number of drugs being...

Differential diagnosis of localized ACLE

Pictures Target Lesions

The diseases, which we may consider in the differential diagnosis with localized ACLE, are acne rosacea, contact dermatitis and photodermatitis, seborrheic dermatitis, dermatomyositis, erysipelas, and delusion of lupus. In its early stages, rosacea may present with a purely erythematous rash on the malar areas that can be very similar to ACLE. The rash may be triggered or aggravated by heat, cold, emotions, and by the ingestion of hot drinks, alcohol, and spicy foods. Differentiating early...

Differential diagnosis of papulosquamous SCLE

Ring Lesion Central Clearing Scale

The diseases, which we may consider in the differential diagnosis with the papulosquamous pattern of SCLE are psoriasis and polymorphic light eruption. 3.3.1. Psoriasis vs papulosquamous SCLE Psoriasis is a chronic inflammatory disorder characterized by the presence of erythematous scaling plaques that in some cases can resemble SCLE. Differential features are a thicker scaling, with a silvery, micaceous appearance Fig. 9 and a different distribution of psoriasis plaques that predominantly...

Treatment of systemic manifestations

Especially in the young male, where the prognosis, is the worst, it is important to start treatment as early as possible. Efficacy of this approach has formally been shown for eye disease Hamuryudan et al., 1997 and in the management of pulmonary artery aneurysms Hamuryudan et al., 2004 . Azathioprine has been formally shown that to be effective in controlling eye disease and some of the extraocular manifestations including skin-mucosa disease, in a double-blind placebo controlled study....