CUTANEOUS LUPUS ERYTHEMATOSUS Araya Keeratimahat, MD 9/9/2014

CASE

History ผู้ป่วยชายไทยอายุ 52 ปี no U/D Admit 28/8/57 CC : ผื่นนูนแดงคันบริเวณลำตัวส่วนบนมากขึ้น 3 เดือน PTA PI : 4 เดือน PTA ผู้ป่วยเริ่มมีผื่นนูนแดงคันบริเวณที่หลังส่วนบน ลามมาที่หน้าอกส่วนบน ลำคอ ใบหน้า หลังหู 3 เดือน PTA ผื่นนูนแดงคันบริเวณลำตัวส่วนบน ใบหน้าและลำคอ ลามมากขึ้น มีผื่นที่แขนทั้ง 2 ข้างและมีที่บริเวณหน้าแข้งทั้ง 2 ข้าง ร่วมกับมีแผลในปาก ไข้ต่ำๆ ไม่มีปวดข้อ ไม่มีปัสสาวะเป็นฟองหรือเป็นเลือด ไม่มีเลือดออกผิดปกติ ไม่มีชักเกร็ง ไม่มีหายใจหอบเหนื่อย

History Past history Hx old pulmonary TB รักษาครบ course 6 mo 3 yr PTA แพ้ยา Penicillin, Ceftriaxone, Tetracycline สูบบุหรี่ 2-3 มวน/วัน * 10 ปี ดื่มเบียร์ตามเทศกาล 1-2 แก้ว หยุดดื่มประมาณ 6 เดือน ปฏิเสธยาชุด/ยาหม้อ/ยาลูกกลอน/ยาสมุนไพร

Physical examination V/S : BT 36°C, PR 72/min, RR 20/min, BP 116/68 mmHg Skin : - multiple well defined annular erythematous plaque coalesce into polycyclic border at scalp and forehead, no hair loss - bilateral well defined erythematous edematous and papules at malar area with apare nasolabial fold and bridge over the nose

Physical examination Skin : - multiple well defined annular erythematous plaque coalesce into polycyclic border with central hypopigmentation and scaling at V shape of neck, upper back, dorsum of both upper extremities and both shins Oral cavity : solitary well defined erythematous annular plaque with central hypopigmentation at hard palate Nail : no periungual telangiectasia

Physical examination HEENT : not pale conjunctivae, anicteric sclerae LN : no lymphadenopathy Lungs : no crepitation Heart : normal S1 S2, no murmur Abd : Soft, not tender Ext : no petichiae, no ecchymosis, no pitting edema Neuro : E4V5M6, motor gr V all

28/8/57

28/8/57

28/8/57

Problem list Cutaneous lupus erythematosus

CUTANEOUS LUPUS ERYTHEMATOSUS

Lupus Erythematosus A group of heterogeneous illnesses that have in common the development of immunity to self-nucleic acids and their associated proteins, with skin-only disease at one end of the spectrum and severe visceral involvement at the other.

Lupus Erythematosus LE as a clinical spectrum ranging from mildly affected patients with only localized DLE skin lesions to those at risk of dying from the systemic manifestations of LE such as nephritis, central nervous system disease, or vasculitis. Skin lesions may be specific to lupus or nonspecific and are seen in other conditions.

Lupus Erythematosus The term “LE-specific” relates to those lesions displaying an interface dermatitis. Cutaneous LE (CLE) is often used synonymously with “LE-specific skin disease”. The three major categories of LE-specific skin disease: acute cutaneous LE (ACLE) subacute cutaneous LE (SCLE) chronic cutaneous LE (CCLE)

EPIDEMIOLOGY Skin disease is the second most frequent clinical manifestation of LE after joint inflammation. Gender Race Associated with SLE ACLE Women > Men (8:1) All, especially dark skin Generalized ACLE 35-60% SCLE White females 50% CCLE (3:2 – 3:1) blacks 15-30%

PATHOGENESIS Interaction between genetic and environmental factor Ultraviolet radiation, medication, viruses This triggers a complex inflammatory cascade of cytokine, chemokine and inflammatory cell responses that include cells recruited to the skin.

PATHOGENESIS Genetic background Genes previously associated with SLE, e.g. TYK2, IRF5 and CTLA4, also confer an increased risk for developing DLE and SCLE19, while mutations in TREX1, which encodes a DNA exonuclease, are associated with familial chilblain lupus

PATHOGENESIS Autoantibodies play a role in SCLE and neonatal lupus : anti-Ro and anti-La. Ro60 plays an important role in cell survival following UVR. Ro52 has a known regulatory role in inflammation, targeting both interferon regulatory factor 3 (IRF3) and IRF8 for degradation.

PATHOGENESIS Ultraviolet radiation Both ultraviolet B (UVB) and ultraviolet A radiation have been implicated in exacerbation of cutaneous LE, although UVB is a more efficient cause of photo-induced changes in the skin.

PATHOGENESIS Medication The drugs that induce CLE can be linked by their photosensitizing properties. It has been cause an increase in keratinocyte apoptosis, exposure of previously intracellular peptides on epidermal cell surfaces, and enhance proinflammatory cytokines such as TNF-α and IFN-α.

Pathogenesis of lupus erythematosus Pathogenesis of lupus erythematosus. A In photosensitive cutaneous LE, ultraviolet radiation (UVA and UVB) triggers cytokine and chemokine production, initiating an immune response. B A lichenoid tissue reaction is the endpoint of a complex cascade that includes activation of dendritic cells, release of interferon (IFN), production of chemokines, and activation of T cells. BMZ, basement membrane zone; CCL, chemokine (C-C motif) ligand; CXCL, chemokine (C-X-C motif) ligand; CXCR, chemokine (C-X-C motif) receptor; HMGB1, high-mobility group box 1; IL, interleukin; ICAM, intercellular adhesion molecule; pDC, plasmacytoid dendritic cell; TNF, tumor necrosis factor; VCAM, vascular cell adhesion molecule

Pathogenesis of lupus erythematosus Pathogenesis of lupus erythematosus. A In photosensitive cutaneous LE, ultraviolet radiation (UVA and UVB) triggers cytokine and chemokine production, initiating an immune response. B A lichenoid tissue reaction is the endpoint of a complex cascade that includes activation of dendritic cells, release of interferon (IFN), production of chemokines, and activation of T cells. a positive feedback loop ultimately results in a lichenoid tissue reaction BMZ, basement membrane zone; CCL, chemokine (C-C motif) ligand; CXCL, chemokine (C-X-C motif) ligand; CXCR, chemokine (C-X-C motif) receptor; HMGB1, high-mobility group box 1; IL, interleukin; ICAM, intercellular adhesion molecule; pDC, plasmacytoid dendritic cell; TNF, tumor necrosis factor; VCAM, vascular cell adhesion molecule

CLINICAL FEATURES Cutaneous Lupus – Three Major Forms Classification Specific skin lesions and not specific lesions. the category of specific cutaneous lesions subdivided into acute cutaneous LE (ACLE) subacute cutaneous LE (SCLE) chronic cutaneous LE (CCLE)

Predominant locations of inflammatory infiltrates in subsets of cutaneous lupus erythematosus. The types of cutaneous lupus erythematosus are: acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE), discoid lupus erythematosus (DLE), lupus erythematosus tumidus (LET) and lupus panniculitis (LEP); the latter three are forms of chronic cutaneous lupus erythematosus (see Table 41.2). The primary locations of the infiltrates are as follows: superficial dermis, ACLE and SCLE; superficial plus deep dermis and periadnexal, DLE; superficial and deep dermis, LET; and subcutaneous fat, LEP. The final diagnosis requires clinicopathologic correlation.

Acute cutaneous lupus erythematosus Localized ACLE has commonly been referred to as the classic butterfly rash or malar rash of SLE. In localized ACLE, confluent symmetric erythema and edema are centered over the malar eminences and bridges over the nose (unilateral involvement with ACLE has been described).

Acute cutaneous lupus erythematosus Localized acute cutaneous lupus erythematosus. Erythematous, slightly edematous, sharply demarcated erythema is seen on the malar areas in a “butterfly”distribution.

Acute cutaneous lupus erythematosus The face, malar area, is most commonly affected, but may be more widespread in distribution The morphology of the lesions ranges from mild erythema to intense edema.

Acute cutaneous lupus erythematosus Acute cutaneous lupus erythematosus (ACLE). The facial erythema, often referred to as a “butterfly rash” may be variable (A), edematous (B) or have associated scale (C). The presence of small erosions can aid in the clinical differential diagnosis Acute cutaneous lupus erythematosus (ACLE). The facial erythema, often referred to as a “butterfly rash” may be variable, edematous or have associated scale. The presence of small erosions can aid in the clinical differential diagnosis

Acute cutaneous lupus erythematosus Transient, follow sun exposure, and resolve without scarring (but sometimes with dyspigmentation). The duration may range from a few hours to several weeks. An association with anti-dsDNA antibodies and lupus nephritis.

Acute cutaneous lupus erythematosus Generalized ACLE presents as a widespread morbilliform or exanthematous eruption often focused over the extensor aspects of the arms and hands and characteristically sparing the knuckles. Acute cutaneous lupus erythematosus (ACLE). The facial erythema, often referred to as a “butterfly rash” may be variable (A), edematous (B) or have associated scale (C). The presence of small erosions can aid in the clinical differential diagnosis

Acute cutaneous lupus erythematosus Well-demarcated patches of erythema with fine overlying scale on the dorsal aspect of the hands, fingers, and periungual areas. Note the characteristic sparing of the knuckles, which are preferentially involved in dermatomyositis Acute cutaneous lupus erythematosus (ACLE). The facial erythema, often referred to as a “butterfly rash” may be variable (A), edematous (B) or have associated scale (C). The presence of small erosions can aid in the clinical differential diagnosis

Acute cutaneous lupus erythematosus Periungual erythema and grossly visible telangiectasia. Acute cutaneous lupus erythematosus (ACLE). The facial erythema, often referred to as a “butterfly rash” may be variable (A), edematous (B) or have associated scale (C). The presence of small erosions can aid in the clinical differential diagnosis

Acute cutaneous lupus erythematosus Rarely ACLE seen that can simulate toxic epidermal necrolysis (TEN). Occurs on predominantly sun-exposed skin and has a more insidious onset. The mucosa may or may not be involved, as in TEN. Acute cutaneous lupus erythematosus (ACLE). The facial erythema, often referred to as a “butterfly rash” may be variable (A), edematous (B) or have associated scale (C). The presence of small erosions can aid in the clinical differential diagnosis

Subacute cutaneous lupus erythematosus SCLE is typically photosensitive, with lesions confined to sun-exposed skin. the midfacial skin is usually spared, while the sides of the face, upper trunk and extensor aspects of the upper extremities are commonly involved

Subacute cutaneous lupus erythematosus Lesions of SCLE may have an annular configuration, with raised red borders and central clearing a papulosquamous presentation with an eczematous or psoriasiform appearance. SCLE lesions often result in dyspigmentation, particularly hypopigmentation or even depigmentation, but do not scar.

Subacute cutaneous lupus erythematosus Subacute cutaneous lupus erythematosus (SCLE). Numerous erythematous annular plaques on the back, some of which have associated white scale. Note the photodistribution.

Subacute cutaneous lupus erythematosus Subacute cutaneous lupus erythematosus (SCLE). Lesions are most commonly seen on the sun-exposed aspects of the upper extremities. The margins of the annular lesions may have scale-crust

Subacute cutaneous lupus erythematosus Approximately 50% of patients with SCLE meet the ACR’s revised criteria for the classification of SLE. The manifestations of severe SLE, such as nephritis, central nervous system disease, and systemic vasculitis, develop in only 10%–15% of patients with SCLE.

Subacute cutaneous lupus erythematosus The risk factors for the development of SLE in a patient presenting with SCLE lesions are: - the papulosquamous type of SCLE, - leukopenia, - high titer of antinuclear antibody (ANA) (>1:640) - anti-dsDNA antibodies

Subacute cutaneous lupus erythematosus variants of SCLE Rowell syndrome (erythema multiforme-like lesions occurring in patients with SLE in the presence of La/SS-B autoantibodies).

Subacute cutaneous lupus erythematosus The prevalence of anti-Ro in SCLE, approximately 70% in a large series (reported range of 60–100%) have anti-Ro antibody. SCLE can overlap with other autoimmune diseases, including Sjögren’s syndrome, rheumatoid arthritis, and Hashimoto’s thyroiditis.

Subacute cutaneous lupus erythematosus SCLE have appeared in patients receiving certain medications, in particular hydrochlorothiazide and terbinafine. The lesions may or may not clear once the medication is discontinued.

Subacute cutaneous lupus erythematosus There has also been the suggestion that SCLE can be associated with internal malignancy (breast, lung, gastric, uterine, hepatocellular, and laryngeal carcinomas as well as with Hodgkin lymphoma).

Chronic cutaneous lupus erythematosus

Chronic cutaneous lupus erythematosus Discoid lupus erythematosus Discoid lesions represent one of the most common skin manifestations of lupus. found most often on the face, scalp and ears, but may be present in a widespread distribution. On occasion, discoid lesions occur on mucosal surfaces, including the lips, nasal mucosa, conjunctivae and genital mucosa. It is unusual for discoid lesions to be present below the neck without lesions also being present above the neck Some patients with discoid lesions exhibit a photodistribution,

Chronic cutaneous lupus erythematosus Discoid lupus erythematosus Early classic DLE lesions typically evolve into sharply demarcated, coin-shaped (i.e., discoid) erythematous plaques covered by a prominent, adherent scale that extends into the orifices of dilated hair follicles. Classic discoid lupus erythematosus. Typical early erythematous plaque on the forehead demonstrating hyperkeratosis and accentuation of follicle orifices It is unusual for discoid lesions to be present below the neck without lesions also being present above the neck Some patients with discoid lesions exhibit a photodistribution,

Chronic cutaneous lupus erythematosus Discoid lupus erythematosus Follicular involvement in DLE is a prominent feature. Keratotic plugs accumulate in dilated follicles that soon become devoid of hair.

Chronic cutaneous lupus erythematosus Discoid lupus erythematosus When the adherent scale is lifted from more advanced lesions, keratotic spikes similar in appearance to carpet tacks can be seen to project from the undersurface of the scale (the “carpet tack” sign). Discoid lesions have the potential for scarring.

Chronic cutaneous lupus erythematosus Discoid lupus erythematosus Active lesions Indurated on palpation Longstanding lesions, hypopigmentation in the central area and hyperpigmentation at the periphery Follicular plugging and adherent scale Atrophic dermal scarring or scarring alopecia

Chronic cutaneous lupus erythematosus Discoid lupus erythematosus The scalp is involved in 60% of patients with DLE. DLE can present on hairbearing skin (scalp, eyelid margins, and eyebrows). DLE causes scarring alopecia has been reported in one-third of patients. It is unusual for discoid lesions to be present below the neck without lesions also being present above the neck Some patients with discoid lesions exhibit a photodistribution,

Chronic cutaneous lupus erythematosus Lupus hair, may be telogen effluvium occurring as the result of flaring systemic disease. the reversible, nonscarring alopecia that patients with SLE often develop during periods of systemic disease activity.

Chronic cutaneous lupus erythematosus Discoid lupus erythematosus DLE characteristically affects the external ear, including the outer portion of the external auditory canal

Chronic cutaneous lupus erythematosus Discoid lupus erythematosus Generalized DLE more commonly associated with underlying SLE and is often more recalcitrant to standard therapy. DLE lesions below the neck most commonly occur on the extensor aspects of the arms, forearms, and hands.

Chronic cutaneous lupus erythematosus Discoid lupus erythematosus DLE lesions can be potentiated by sunlight exposure but to a lesser extent than ACLE and SCLE lesions. DLE, can be precipitated by any form of cutaneous trauma (i.e., the Koebner phenomenon).

Chronic cutaneous lupus erythematosus Discoid lupus erythematosus The relationship between classic DLE and SLE 5% of patients presenting with classic DLE lesions subsequently develop of SLE Patients with generalized DLE have higher rates of immunologic abnormalities, a higher risk for progressing to SLE and a higher risk for developing more severe manifestations of SLE. one-fourth of patients with SLE develop DLE lesions tend to have less severe forms of SLE.

Chronic cutaneous lupus erythematosus Hypertrophic DLE a rare variant hyperkeratosis in classic DLE lesions is greatly exaggerated. The extensor aspects of the arms, the upper back, and the face are the areas most frequently affected.

Chronic cutaneous lupus erythematosus Hypertrophic DLE Patients with hypertrophic DLE probably do not have a greater risk for developing SLE than do patients with classic DLE lesions

Chronic cutaneous lupus erythematosus Mucosal DLE ≈25% of patients with CCLE. The oral mucosa is most frequently affected; however, nasal, conjunctival, and genital mucosal surfaces can be targeted.

Chronic cutaneous lupus erythematosus Mucosal DLE In the mouth, the buccal mucosal surfaces are most commonly involved, with the palate, alveolar processes, and tongue. Central depression often occurs in older lesions, and painful ulceration can develop.

Chronic cutaneous lupus erythematosus Mucosal DLE Chronic DLE plaques also appear on the vermilion border of the lips. DLE can present as a diffuse cheilitis, especially on the more sun-exposed lower lip. Conjunctival DLE lesions more affect the lower lid and the permanent loss of eyelashes and ectropion can developed.

Chronic cutaneous lupus erythematosus LE Profundus/ LE Panniculitis LE Panniculitis : rare form of CCLE typified by inflammatory lesions in the lower dermis and subcutaneous tissue. LE profundus : ≈70% of patients have DLE lesions overlying the panniculitis lesions The head, proximal upper arms, chest, back, breasts, buttocks, and thighs are the sites frequently affected.

Chronic cutaneous lupus erythematosus LE Profundus/LE Panniculitis Typical subcutaneous lesions present as firm nodules, 1–3 cm in diameter. The overlying skin often becomes attached to the subcutaneous nodules and is drawn inward to produce deep, saucerized depressions.

Chronic cutaneous lupus erythematosus LE Profundus/LE Panniculitis 50% of patients with LE profundus/ panniculitis have evidence of SLE but tend to be less severe.

Chronic cutaneous lupus erythematosus Chilblain LE Purple-red patches, papules, and plaques on the toes, fingers, and face, which are precipitated by cold, damp climates. Chilblain LE appears to be associated with anti-Ro/ SS-A antibodies and is linked to Raynaud’s phenomenon in many cases. ≈ 20% of patients presenting with chilblain LE later develop SLE

Chronic cutaneous lupus erythematosus Chilblain lupus. Violaceous plaques, some with scale, on toes. If there is a family history of this disorder, the possibility of mutations in TREX1, which encodes a DNA exonuclease, can be considered.

Chronic cutaneous lupus erythematosus Lupus Erythematosus Tumidus The lesion characterized by induration and erythema but no scale or follicular plugging. The epidermis appears to be uninvolved in the disease process, although there is an intense dermal inflammatory infiltrate. Some patients with SLE, most patients with LET have a negative ANA and a benign disease course.

Chronic cutaneous lupus erythematosus Lupus Erythematosus Tumidus LE tumidus lesions may be the same as the “urticarial plaques” described in lupus patients.

Chronic cutaneous lupus erythematosus Lupus Erythematosus Tumidus Lesions are most common on the face and can be quite common on the trunk. The lesions tend to resolve without scarring or atrophy.

Neonatal lupus erythematosus A neonatal form of SCLE may occur in infants whose mothers have anti-Ro autoantibodies. Almost 100% of babies with NLE have anti-Ro antibodies. Annular erythematous plaques with predilection on the face, especially the periorbital region and scalp.

Neonatal lupus erythematosus The major extracutaneous findings are congenital heart block (with or without cardiomyopathy), hepatobiliary disease and thrombocytopenia. Cardiac NLE has a mortality rate of approximately 20%, and about two-thirds of children require pacemakers.

Nonspecific Cutaneous Lesions Vascular lesions are common in patients with LE, particularly in those who have systemic disease. Livedo reticularis, thromboses, ulcerations, and lesions resembling Degos’ disease have each been associated with antiphospholipid antibodies. Sclerodactyly, calcinosis and rheumatoid nodules, some patients with these findings may have overlap syndromes rather than classic lupus. Alopecia often occurs as a result of scarring discoid lesions increased likelihood of alopecia areata

LABORATORY TESTS The laboratory features of ACLE are those associated with SLE (high-titer ANA, anti-dsDNA, anti-Sm, and hypocomplementemia). The laboratory markers for SCLE are the presence of anti-Ro/SS-A (70%–90%) and, less commonly, anti-La/SS-B (30%–50%) autoantibodies.

LABORATORY TESTS

LABORATORY TESTS

PATHOLOGY Acute cutaneous LE showing interface dermatitis with vacuolization of basal keratinocytes and sparse superficial lymphoid infiltrates. Histologic features of cutaneous lupus erythematosus (LE). A Acute cutaneous LE showing interface dermatitis with vacuolization of basal keratinocytes and sparse superficial lymphoid infiltrates. B Chronic discoid LE showing focal interface dermatitis and dense perivascular and periadnexal lymphoid infiltrates throughout the entire dermis. A thickened basement membrane is a characteristic finding and can be highlighted by PAS staining (insert).

PATHOLOGY Chronic discoid LE showing focal interface dermatitis and dense perivascular and periadnexal lymphoid infiltrates throughout the entire dermis. Histologic features of cutaneous lupus erythematosus (LE). A Acute cutaneous LE showing interface dermatitis with vacuolization of basal keratinocytes and sparse superficial lymphoid infiltrates. B Chronic discoid LE showing focal interface dermatitis and dense perivascular and periadnexal lymphoid infiltrates throughout the entire dermis. A thickened basement membrane is a characteristic finding and can be highlighted by PAS staining (insert).

Immunopathology of Lesional Skin Direct immuno-fluorescence of cutaneous lupus. Granular deposits of IgM are present at the dermal–epidermal junction within lesional skin. Direct immunofluorescence of cutaneous lupus. Granular deposits of IgM are present at the dermal–epidermal junction within lesional skin. Antibody deposits at the dermal–epidermal junction are the most characteristic immunohistologic finding in lesions of cutaneous lupus and normal skin of patients with systemic lupus erythematosus

Systemic lupus erythematosus

Treatment Topical Therapy Topical or intralesional corticosteroids are a mainstay of therapy. Discoid lesions may be appropriate to use high-potency corticosteroids on the face. Superpotent topical class I agents, such as clobetasol propionate 0.05% or betamethasone dipropionate 0.05%, produce the greatest benefit in CLE.

Treatment Topical Therapy Twice-daily application of the superpotent preparations to lesional skin for 2 weeks followed by a 2-week rest period can minimize the risk of local complications such as steroid atrophy and telangiectasia Patients should be instructed about the risks and benefits of therapy, the need to limit the application to affected areas.

Treatment Topical Therapy Particularly in active discoid lesions and LE tumidus lesions, intralesional triamcinolone, often given in a concentration of 4–5 mg/ml, can be very effective. The injections may be repeated monthly while the lesions are active.

Treatment Topical Therapy Topical Calcineurin Inhibitors. Pimecrolimus 1% cream and tacrolimus 0.1% ointment. Alternatively, a topical calcineurin inhibitor can be used daily during the 2-week rest period from topical corticosteroids.

Treatment Topical Therapy Topical Calcineurin Inhibitors. A double blind, placebo controlled pilot study showed that pimecrolimus 1% cream had equal efficacy with betamethasone valerate 0.1% cream in treating facial DLE. Topical tacrolimus 0.3% compound in clobetasol propionate 0.05% for recalcitrant CLE.

Treatment Topical Therapy SUN PROTECTION. Avoid direct sun exposure, wear tightly woven clothing and broad brimmed hats, and regularly use broad-spectrum, water-resistant sunscreens SPF ≥30 with an efficient UVA blocking agent such as a photostabilized form of avobenzone, micronized titanium dioxide, micronized zinc oxide.

TREATMENT Systemic Therapy Antimalarials. antimalarials can be effective for ≈ 75% of patients with CLE. The risks of retinal toxicity should be discussed with the patient, and pretreatment ophthalmologic examination should be performed.

TREATMENT Systemic Therapy about 2–3 month delayed onset of therapeutic benefit. If no response is seen after 8–12 weeks, quinacrine hydrochloride can be add

TREATMENT Systemic Therapy If, after 4–6 weeks, adequate clinical control has not been achieved, consideration should be given to replacing the hydroxychloroquine with chloroquine diphosphate 3 mg/kg to prevent retinopathy. Hydroxychloroquine and chloroquine should not be used simultaneously because of enhanced risk for retinal toxicity.

TREATMENT Systemic Therapy Systemic corticosteroids patients who have especially severe and symptomatic skin disease, intravenous pulse methylprednisolone has been used. In less acute cases, moderate daily doses of oral glucocorticoids (prednisone, 20–40 mg/day, given as a single morning dose) can be used as supplemental therapy during the loading phase of therapy with an antimalarial agent.

TREATMENT Systemic Therapy When there is a mild degree of major organ involvement, corticosteroids are the primary treatment, but for moderate to severe involvement, pulse cyclophosphamide +/− pulse corticosteroids are often recommended. Immune response modifiers (biologic therapies) are reserved for refractory disease.

TREATMENT Systemic Therapy

TREATMENT Systemic Therapy

Best Practice & Research Clinical Rheumatology 27 (2013) 391–404

PROGNOSIS AND CLINICAL COURSE The prognosis for patient with ACLE is dictated by the pattern of the underlying SLE. Both 5-year (80%–95%) and 10-year (70%–90%). ≈ 15% of the patients with SCLE develop active SLE, including lupus nephritis.

PROGNOSIS AND CLINICAL COURSE CCLE with localized DLE have only a 5% chance of subsequently developing clinically significant SLE disease activity. Most patients with untreated classic DLE lesions suffer indolent progression to large areas of cutaneous dystrophy and scarring alopecia that can be psychosocially devastating and occupationally disabling.

Referrence LEE AL. and Werth VP. Lupus Erythematosus. In: Bolognia JL, Jorizzo JL, Schaffer JV, et al. Dermatology 3rd ed. Elsevier 2012: 615-29. Okon LG and Werth VP. Best Practice & Research Clinical Rheumatology 27 (2013) 391–404. Petri M, et al. Arthritis and Rheumatism. Aug 2012. www.rheumtutorcom/2012-slicc-sle-creteria Sontheimer RD and Costner MI. Lupus Erythematosus. In: Goldsmith LA, Gilchrest BA, Paller AS, Leffel DJ, Wolff K, editors. Fitzpatrick’s dermatology of general medicine. 8th ed. New York: Mcgraw-Hill 2012: 1909-26.

Investigation CBC Hb 12.0 g% Hct 36.6% WBC 3900 cell/mm3 PMN 57% L 25% (lymphocyte 975 cells) Mono 15% Eo 2% B 1% Plt 148000 UA protein negative WBC 0-2 RBC 0-1 BUN 11 Cr 1.2 Na 138 K 3.54 Cl 104 Co2 27.4 Ca 8.58 Mg 0.8 P 2.63 AST 84 ALT 16 ALP 85 TB 0.44 DB 0.27 Alb 4.0 HBSAg negative HCV Ab negative

Investigation FANA Positive 1:640 (speckle, nucleolar) Anti-SSA (RO) negative, anti-SSB (La) negative, anti DNA negative, anti Sm negative Skin biopsy : basal vacuolization, interface dermatitis with lymphocyte DIF skin lesion : positive IgM colloid body and positive fibrinogen at DEJ discontinuous pattern

Diagnosis Diagnosis : SLE 1. cutaneous LE 2. oral ulcer 3. leukopenia and lymphopenia 4. ANA positive

Treatment Start Hydroxychloroquine (200) 1*2 oral pc Potent topical corticosteroid Sunscreen

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