Cutanous Manifestation of Lupus Erythematosus. Presented By: Dr. Naif S. Al Shahrani Salman Bin Abdaziz university

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1 Cutanous Manifestation of Lupus Erythematosus Presented By: Dr. Naif S. Al Shahrani Salman Bin Abdaziz university

2 A 50-year old lady, who is otherwise healthy, presented to the dermatology clinic with three months history of: - Mild Joint pain. - Itchy skin eruption on palms, soles & lower extremities.

3 There was no history of :- Photosensitivity. Malar rash or oral ulcer. Using any type of medication (including herbal) Other systemic involve. Family Hx with similar illness.

4 ON Examination Erythematous annular plaques with scales over the periphery. Distributed over palms, soles, knees, arms & thighs. Face & Mucus membranes free. Hair / Nail examination with in normal.

5 Investigation

6 CBC: Hgb 12 g/l U/E & LFT: Normal

7 Serology ANA 1:160 Anti ds-dna 22.5 H Anti (Ro) 34.2 H Anti (La) < 3.1 Anti Smith < 3.1 Anti SCL-70 < 3 Anti JO1 < 3.1 Anti RNP 6.7

8 Differential Diagnosis Psoriasis SLE Secondary Syphilis MF

9 Skin Biopsy

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22 Skin Biopsy 3 mm punch biopsy was taken from: Left Thigh ( consistent with lupus dermatitis) Coupled with the clincal presentation & serologic profile ( consistent with subacute lupus dermatitis)

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24 Lupus Erythematosus

25 Objectives Epidemiology of cutaneous lupus. Review classification of cutaneous lupus Morphology Pictures Associations Treatments

26 Epidemiology Prevalence 14 to 68 per people. F:M = 3:1 All races affected. African Americans discoid lesions, photosensitivity

27 Cutaneous Lupus Classified Cutaneous Lupus Erythematosus (CLE) LE Specific LE Non Specific Acute Cutaneous (ACLE) Subacute Cutaneous LE (SCLE) Chronic Cutaneous LE (CCLE) Numerous manifestations

28 Why Classify? Type of skin involvement can reflect underlying pattern of SLE. Acute, sub-acute and chronic refer to severity of disease not necessarily duration of disease. ACLE almost always associated with SLE flare. CCLE often in the absence/mild SLE. SCLE somewhere in between.

29 ACLE Variants: Localized vs Generalized Localized Lesions: erythematous plaques, papules, macules +/- telangiectasia, +/- dyspigmentation Distrbution: forehead, V area of neck, malar rash sparing nasolabial folds Photosensitive Typically non scarring Transient: days to weeks Associated with facial edema

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33 DDx Rosacea Sunburn Drug induced hypersensitivity. Seborrheic dermatitis

34 Generalized Morbilliform. ACLE Distribution: widespread, often focused over extensor aspects of arms & hands, characteristically sparing knuckles. Photosensitive, non scarring, transient. DDx Viral drug rash Eczema Drug eruption TEN

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37 ACLE Serology High ANA titre Anti-dsDNA, Anti-Sm Low complement Epidemiology F:M 8:1

38 SCLE Lesions - 2 major manifestations: Annular Red raised borders with central clearing. Papulosquamous. red, raised, well demarcated, scale. Distribution: upper back, shoulders, extensor aspects of arms, V area of neck, less commonly the face. Photosensitive. No scarring but can resolve with long lasting telangiectasias and hypopigmentation.

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42 DDx Annular Tinea. Granuloma annulare. Papulosquamous Psoriasis. Eczema. Cutaneous T cell Lymphoma.

43 SCLE % may develop into ACLE or DLE. SCLE vs ACLE: last longer, heal with more pigmentary change, less edematous, more hyperkeratosis, distribution. SCLE vs DLE: less pigmentary change, typically does not scar, no follicular plugging and less adherent scale.

44 SCLE Can be induced/exacerbated drugs: Cardiac (Ca Channel blockers, ACEI, spironolactone ). Antifungal/antibacterial (terabinfine,griesofulvin). Anti-reflux (lansoprazole, ranitidine) Antinflammatory (naproxen) Other (parenteral gold, carbamazepine)

45 Serology Anti-Ro ~ 70-90% Anti-La ~ 30-50% ANA ~ 60-80% RF ~ 33 % Epidemiology Present in 7-27 % of patients with SLE Primarily white females in 5 th decade SLE develop in ~ %

46 Neonatal lupus erythematosus SCLE like lesion but mainly over the face (preiorbital region). Infants whose mother has anti-ro autoantibodies. Resolved without scaring. Extracutanous findings are congenital heart block, hepatobiliary disease & thrombocytopenia.

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48 Chronic cutaneous lupus erythematosus (ccle)

49 CCLE: (DLE) Variants: localized, generalized & hypertrophic Localized: neck & above. Generalized: above & below neck. Hypertrophic: often over extensor arms with thick scaling at perphery. Lesions: Coin shaped (discoid) erythematous, indurated plaques Adherent scale. Folliclular plugging. Scarring alopecia. Heals with scarring: hypopigmentation at centre, hyperpigmentation at periphery. Squamous cell carcinoma may develop in scar; rare. Distribution: Scalp, ears, face, V of neck, mucosal involvement (25 %). Can be potentiated by sun but to a lesser extent as compared with ACLE & SCLE.

50 DLE Epidemiology % of SLE patients 5 % presenting with DLE progress to SLE Most common in females (M:F = 3:2) at years of age

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54 DDx Polymorphous light eruption Cutaneous Lymphoma B cell T cell Sarcoidosis Lichen planus

55 Ccle:(Lupus erythematosus tumidus) Indurated erythema but no scale or follicular plugging. Commonly over the face & trunck. Described as ( urticarial plaques) Resolve without scaring or atrophy.

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57 CCLE: (lupus panniculitis) Tender subcutaneous nodules & Indurated plaques. Mainly over the face and upper arms. Precedes the onset of systemic lupus erythematosus.(10-15% of patient) Some with discoid lesion overlying the panniculitis,reffered as( lupus profundus).

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59 CCLE:(CHILBLAIN LUPUS) Also knowen as (SLE pernio). Red or dusky purple papules & plaqes on fingers,toes, nose & elbow. Exacerbated by cold. ANA titer is positive.

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61 Other variants

62 Bullous L.E Blistering eruption that consists of vesicles & bullae whose histopathology often resemble dermatitis herpetiformis. Autoantibodies against type 7 collagen which developed in patient with systemic L.E.

63 CUTANEOUS LUPUS MUCINOSIS Occurs in 1.5% of patient with L.E. Asymptomatic skin-colored papules & nodules. Common in back & v- area of the chest. 75% of L.E patient who develop it have systemic involvement ( mainly renal) Mucin abounds in upper and mid dermis with mild preivascular lymphocytic infiltrate. No L.E epidermal changes seen.

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67 Treatment Investigate for SLE. Avoid potentially photosensitizing drugs. Photoprotection. Behavior modification Sunscreen

68 Treatment Localized Most commonly SCLE & DLE Local glucocorticoids (GC) Topical Degree of potency selected based on site of involvement GC solutions best for scalp Often need to treat aggressively to obtain results Intralesional Used for DLE most commonly Thick, solitary lesions unresponsive to topical agents

69 Systemic Most commonly used with ACLE and when topicals fail in SCLE or DLE Antimalarials Hydroxychloroquine sulfate (Plaquenil) 6mg/kg Chloroquine phosphate (Aralen) 4 mg/kg Antimalarial Resistant Cutaneous Disease Disease resistant to antimalarials often resistant to other modalities Low dose systemic corticosteroid effective for malar rash but rarely for DLE & partially for SCLE Azathioprine,MTX,Mycophenolate mofetil Oral Retinoids &Thalidomide

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