The most common adverse reaction from this therapeutic agent is GI side effects, including nausea, vomiting, diarrhea, and abdominal cramps. Cutaneous involvement in SLE is classified as acute, subacute, and chronic cutaneous lupus erythematosus. The treatment goal for SCLE is to decrease the inflammation in active lesions and hopefully avoid severe In terms of genetics, genes encoding for cytokines, cytokine receptors, adhesion molecules and apoptosis genes are felt to contribute to the development of lupus erythematosus. Subacute cutaneous lupus erythematosus (SCLE) The rash usually appears as red, non-itchy patches on sun-exposed areas such as the upper chest and back and arms. Annular lesions, on the other hand, are ring-shaped and a bit scaled on the edge of the lesions. Subacute cutaneous lupus may be a sign of systemic lupus, but it can also develop on its own. Risk factors for this side effect include drug interactions (trimethoprim/sulfamethoxazole [TMP/SMX] and NSAIDS), renal insufficiency, older age (>65) and no folate supplementation. The lesions occur most commonly on the sun-exposed areas of the arms, shoulders, neck, and body. Please login or register first to view this content. Inherited deficiencies of complement also play a role in the risk of CLE development. Subacute cutaneous lupus erythematosus (SCLE) — This kind will cause sores on parts of the skin that have been exposed to the sun like the upper trunk and arms. Maintenance doses as low as 50mg may be used once disease is stable. vol. (Courtesy of Bryan Anderson, MD). Anti-La/SSB may also be present, but is less frequent. Given the chronic and recurrent nature of CLE, including SCLE, use of steroids should be avoided. The annular-polycyclic lesions variant may be confused with figurate erythema or a superficial dermatophyte infection (Figure 2). Subacute cutaneous lupus erythematosus (SCLE) can rarely be a paraneoplastic phenomenon, appearing simultaneously with the onset of a cancer, resolving with successful cancer treatment and relapsing with recurrence of the malignancy, thus fulfilling McLean’s criteria for paraneoplastic dermatoses.1–6 While paraneoplastic SCLE is reported in association with … Both of these therapies have limited severe adverse reactions. (The distribution and type of lesions, systemic features and immunologic findings between CCLE and SCLE are discussed. subacute cutaneous lupus erythematosus - this is an unpleasant disease. All patients should be encouraged to stop smoking and should begin a smoking cessation program. Your use of this website constitutes acceptance of Haymarket Media’s Privacy Policy and Terms & Conditions. SCLE is a subtype of CLE presenting as symmetric, non-scarring photosensitive erythematous rash usually over syn-exposed areas like face, neck, arms, upper back, and shoulders. - Full-Length Features For instance, patients deficient in C1q are seen in photosensitive-LE like eruptions. The main types of lupus are discoid lupus erythematosus (DLE), subacute cutaneous lupus erythematosus (SCLE) and systemic lupus erythematosus. If significant systemic symptoms of SLE are suspected, a rheumatologist should be involved in the case. J Invest Dermatol. 2010. pp. (An open-label clinical trial of azathioprine use in 12 patients with SCLE, CCLE and vasculitis. 99. A potential for hepatotoxicity, with long-term use, and pulmonary toxicity are important considerations. If the ANA is elevated (> 1:160) or a patient has symptoms suggestive of systemic lupus, further testing is warranted. Subacute cutaneous lupus erythematosus (SCLE) is a clinically distinct form of CLE that presents as a highly photosensitive annular-polycyclic or papulosquamous eruption distributed symmetrically on sun-exposed areas (Figure 1). UVB, UVA and visible light can contribute to induction of skin lesions. 2005. pp. Given that lupus is more common in women of childbearing age, estrogen is felt to contribute to the disease pathogenesis. ), Close more info about Subacute Cutaneous Lupus Erythematosus (SCLE). SCLE usually appear on the chest area, upper back and neck but can also be develop on the face and arms. Renal function can be evaluated once a year, or sooner if renal dysfunction is suspected. The papulosquamous variant, which is more common, appears as psoriasiform plaques and can be misdiagnosed as photosensitive psoriasis. However, SCLE is most often diagnosed in middle-aged women. Their role in local induction of clinical lesions is unknown. Mainstay therapies for the majority of SCLE subsets include topical therapies and antimalarials. Papulosquamous lesions are red scaly patches that resembles psoriasis. Optimal Therapeutic Approach for this Disease, Unusual Clinical Scenarios to Consider in Patient Management, Discoid Lupus Erythematosus (DLE, Chronic Discoid Lupus Erythematosus), Sun protection and avoidance     Broad spectrum ,UVA and UVB,     sunscreen protectionSmoking cessation     TopicalTopical steroids, Class I or II; lower strength for the faceTopical calcineurin inhibitors with or without topical steroid     Tacrolimus 0.1% ointment     Pimecrolimus 1% cream     SystemicAntimalarials:     Hydroxychloroquine 6.0 to 6.5mg/kg/day IBW     Hydroxychloroquine + quinacrine 100mg daily     Chloroquine <3.5mg/kg/day IBW +/- quinacrine 100mg dailyThalidomide 50 to 200mg/dayDapsone 50 to 150mg/dayPrednisone 0.5 to 0.75mg/kg/dayMethotrexate 5 to 25mg/weekMycophenolate Mofetil 2 to 3g/dayAzathioprine 1 to 2.5 mg/kg/day. In cases refractory to antimalarials, dapsone may be an adjunctive treatment in SCLE at dosages of 25 to 150 mg daily. Azathioprine is an immunosuppressant that has been extensively studied in lupus erythematosus as a steroid-sparing agent. Mild systemic disease, such as joint complaints and serologic abnormalities, are common. Both are dose dependent and occur, to some degree, in all patients who take dapsone. Furthermore, after clearance of SCLE lesions, therapies should be reduced to the lowest effective dose, or discontinued. There are two main forms of SCLE: Papulosquamous SCLE occurs in approximately two thirds of cases and consists of red scaly patches. Treatment options are summarized in the Table 1. However, patients with refractory or widespread disease may need treatment with therapies that carry higher side effect risks. Subacute cutaneous lupus erythematous. The lesions usually do … Thalidomide can also be used in short courses for disease flare in patients on maintenance antimalarials. Ideal body weight is calculated as follows: 45.5kg (use 50kg for males) + 2.3 kg for each inch over 5 feet; or 45.5kg + 2.3kg * (height [inches]-60). Use in cases of severe widespread disease, while awaiting antimalarials or other less toxic therapies to take effect is reasonable. Prior to the use of azathioprine, a thiopurine methyltransferase (TPMT) enzyme level can be performed, particularly if doses above 50mg a day are used initially. Discoid lupus erythematosus (DLE) and subacute cutaneous lupus erythematosus (SCLE) are variants of cutaneous lupus erythematosus (LE) that may occur independently or as manifestations of systemic lupus erythematosus (SLE). Antimalarials may be less effective in smokers, and smokers are more likely to have skin disease that is refractory to all therapies. The blurred vision and corneal deposition that may occur is reversible. Of note, abnormal sensory nerve action potentials have been found in clinically asymptomatic patients. ), Herrero, C, Bielsa, I, Font, J, Lozano, F, Ercilla, G, Lecha, M. “Subacute cutaneous lupus erythematosus: clinicopathologic findings in thirteen cases”. We wish you a cure and never get sick of this disease! As a result, all patients with CLE and SLE should be counseled on smoking cessation. Side effects include GI symptoms of nausea, vomiting and abdominal cramping. Cutaneous lupus erythematosus (LE) may present in a variety of clinical forms. The photos of subacute cutaneous lupus erythematosus below are not recommended for people with a weak psyche! You can help Wikipedia by expanding it. Home » Decision Support in Medicine » Dermatology. SCLE: Superficial and deep perivascualar lymphocytic infiltrate with thickened basement membrane and interface changes (Courtesy of Bryan Anderson, MD). List of human leukocyte antigen alleles associated with cutaneous conditions, "Subacute Cutaneous Lupus Erythematosus (SCLE): Background, Etiology, Epidemiology", Seborrheic keratosis with squamous atypia, Palisaded neutrophilic and granulomatous dermatitis, Lupus erythematosus–lichen planus overlap syndrome, Morphea–lichen sclerosus et atrophicus overlap, https://en.wikipedia.org/w/index.php?title=Subacute_cutaneous_lupus_erythematosus&oldid=1013002420, Creative Commons Attribution-ShareAlike License, This page was last edited on 19 March 2021, at 13:59. Baseline laboratory tests include CBC with differential, complete metabolic panel (liver function tests and renal function), urinalysis and G6PD level. Overall, this complex inflammatory cascade between necrosis, apoptosis, autoantibodies, T and B cells, and vascular changes leads to the development of cutaneous lupus erythematosus. We hope you’re enjoying the latest clinical news, full-length features, case studies, and more. Unlike DLE, SCLE is uncommon in African Americans. However, dyspigmentation with hyper- or hypopigmentation can occur and may persist for several months. ), (A review of thirteen cases of SCLE reveal that histopathologic findings of many epidermal colloid bodies and severe epidermal necrosis are seen clinically in patients with annular lesions, anti-Ro antibodies and human leukocyte antigen-DR3. Lupus. - And More, (A comprehensive discussion of medications that may cause SCLE. Patients with SCLE should be aware that there is a risk of progression to SLE, but that the diease is typically mild. ), (The distribution and type of lesions, systemic features and immunologic findings between CCLE and SCLE are discussed. Subacute cutaneous lupus erythematosus (SCLE) Photosensitive cutaneous eruption lasting longer than ACLE but without scarring 10%-15% of patients go on to have systemic findings Inflammatory infiltrate seen in the upper dermis on biopsy Who is at Risk for Developing this Disease? All rights reserved. Cutaneous lupus erythematosus has been classified into three major subtypes: acute cutaneous lupus, subacute cutaneous lupus erythematosus, and chronic cutaneous lupus erythematosus, with discoid lupus being the most common subtype (3). This activity will discuss subacute cutaneous lupus erythematosus (SCLE). [2], Characteristically the lesions appear in sun-exposed areas such as the vee of the neckline or the forearms, but not the face. Those patients with SCLE show less internal organ involvement. Methotrexate (MTX) in lupus erythematosus can be used in doses of 5 to 25mg weekly. Enjoying our content? cutaneous lupus erythematosus (discoid lupus erythema- tosus; DLE) were not excluded as long as the SCLE type of lesion was the major feature of the cutaneous involvement. Annular-polycyclic patches. Patients normally will experience a 2g/dL drop in hemoglobin, but greater drops below 10g/dL necessitate an adjustment of dose. vol. Hypersensitivity reaction, aseptic meningitis and increased cancer risk are also known adverse reactions. Quinacrine can only be obtained at compounding pharmacies. Prednisone should not be used alone, as lesions recur once prednisone is stopped. ), Black, DR, Hornung, CA, Schneider, PD, Callen, JP. Pediatric dermatol 20: 31-34; Black DR et al (2002) Frequency and severity of systemic disease in patients with subacute cutaneous lupus erythematosus. Don’t miss out on today’s top content on Dermatology Advisor. Etiology. The majority of patients have a positive anti-nuclear antibody (ANA) and anti-Ro/SSA. Lupus is a long-term condition that causes joint pain, skin rashes and tiredness. Laboratory tests should be checked 2 weeks after starting therapy, and 2 weeks after increases in dose. 162. The use of broad spectrum sunscreens to cover the UVB and UVA spectrum is necessary. However, a form of publicationbias has likely contributed to this shift in reporting.There is a need for additional large, populationbasedstudies in this area. Subacute Cutaneous Lupus (SCLE). Serious central nervous system and progressive renal disease are uncommon, and SCLE is felt to have a good prognosis. Frequent CBCs are important to monitor for this adverse reaction, and all patients should be on folate supplementation. Thalidomide is also an option for patients who are unable to tolerate antimalarials. Treatment with hydroxychloroquine cleared the cutaneous lesions. All patients newly diagnosed with CLE should be counseled on the specific disease course, including any potential risk for scarring and disfigurement. The response to therapy and management of azathioprine dose is discussed. Established dyspigmentation will not resolve with the above treatments, but may slowly improve over time. Lupus erythematosus (LE) comprises an uncommon group of connective tissue disorders. This is also a more cost-effective way to use specific autoantibody tests. SCLE is more female predominant than discoid lupus. Antimalarials are recommended as first-line systemic therapy for CLE and SLE given its effectiveness in prevention and treatment of symptoms, such as photosensitivity, acute malar rash, DLE, oral ulcers, alopecia, arthritis, pleuritis, and pericarditis. Cigarette smokers are more likely to develop CLE and SLE and suffer from more severe disease. The female to male ratio is 8:1 in SCLE compared with nearly 1:1 in discoid lupus. vol. It comprises 10–15% of cutaneous LE presentations. To cut a long story short I was diagnosed -via letter- some months ago by a dermatologist as having subacute cutaneous lupus, (my main symptom is a photosensitive raised rash, in addition to some join pain plus fatigue) When I went to my GP for some clarification with reference to this diagnosis, she referred me on to a rheumatologist, due to joint pains. Cutaneous lupus erythematosus, including subacute cutaneous lupus, is an autoimmune disease felt to be due to an interplay of genetics, hormones and environment. However, ANA is not specific for SCLE or SLE, a small percentage of the general population will be positive, and ANA positivity increases with increasing age. After antimalarials, there is no one agent that is superior in the treatment of SCLE lesions. vol. Maintenance doses of 1.5 to 3g a day can be used safely. ), (The author discusses the pathogenesis of SCLE from susceptibility genes to loss of tolerance. If antimalarials are ineffective, thalidomide can be used as a second-line agent together with antimalarials. Of note, thalidomide is effective for SCLE and associated arthralgias, but has little effect on the visceral symptoms associated with SLE. Subacute cutaneous lupus erythematosus is a clinically distinct subset of cases of lupus erythematosus that is most often present in white women aged 15 to 40, consisting of skin lesions that are scaly and evolve as poly-cyclic annular lesions or plaques similar to those of plaque psoriasis. The next step is to provide patients with therapeutic modalities that minimize disease progression and improve treatment response. Products with helioplex, mexoryl and physical blockers (titanium dioxide, zinc oxide) offer the broadest protection. An initial ANA, complete blood count (CBC) and urinalysis is sufficient for those without other symptoms. On a similar note, the role of smoking in disease severity must be stressed at the initial visit. Useful tables of the most frequent medications are included. 251-7. (This article compares systemic and serologic findings in patients with SLCE to those with SLE. Tapering prednisone as tolerated once the SCLE lesions are stable is recommended. SCLE is associated with the extended haplotype HLA DRB1*0301-B08. It may cause yellow discoloration of the skin. Consideration of an age-appropriate malignancy workup may be warranted in those cases that are either not responding to standard therapy or in the presence of systemic symptoms or signs that might suggest that the patient has a malignancy. The dose can be increased by 25mg every 2 weeks with a goal of achieving a range between 2 to 3mg/kg/day. [4], Lesions of SCLE may have an annular (shaped like a ring) configuration, with raised red borders and central clearing.[5][6]. A CBC and liver function tests should be performed every 2 weeks while the dose is being adjusted, then every month for the first year, and then every 3 months. The retinopathy associated with antimalarials (chloroquine) may be irreversible. A starting dose of 50mg nightly of thalidomide is recommended and may be increased to normally 100mg at night over 6 to 8 weeks for peak effect, assuming tolerable side effects. SCLE lesions are described as having a scaly red … Thus, when ascending the therapeutic ladder, individualizing therapy for patients based on their co-morbidities is necessary. (A review of thirteen cases of SCLE reveal that histopathologic findings of many epidermal colloid bodies and severe epidermal necrosis are seen clinically in patients with annular lesions, anti-Ro antibodies and human leukocyte antigen-DR3. ACLE may be localized (most often as a malar or ‘butterfly’ rash) or generalized. Typically, hydroxychloroquine is started at 200mg to 400mg a day. Callen, JP. Autoimmunity Reviews. Cutaneous lupus erythematosus (cutaneous LE) includes three subsets of LE-specific skin diseases: acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE), and chronic cutaneous lupus erythematosus (CCLE) (table 1). MMF typically takes approximately 4 weeks to take effect. After 8 to 12 weeks, if improvement is not satisfactory, addition of quinacrine 100mg daily may be added. 1057-62. James, William; Berger, Timothy; Elston, Dirk (2005). Thalidomide is known to be efficacious in SCLE, but its utility in clinical practice is limited by its toxicities. Topical calcineurin inhibitors are effective in SCLE and offer a decreased risk of telangiectasia development compared with topical steroids. ANA and anti-dsDNA can be seen in some patients with SCLE. In North America, SCLE is more common in Caucasians than other ethnicities. High titer anti-Ro/SSA and anti-La/SSA are a common finding in SCLE. Thalidomide produces relatively rapid improvement, as soon as 2 to 3 weeks, and clinical remission is typically achieved within 8 weeks.