Connective Tissue Diseases Dr. Vinitha Varghese Panicker Associate Professor, Department of...

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Lupus Erythematosus (LE)- Types Cutaneous lupus erythematosus is classified into three subtypes: Acute cutaneous lupus erythematosus (ACLE):  Malar rash, Morbilliform rash, bullous lesions Subacute cutaneous lupus erythematosus (SCLE):  Annular, Papulosquamous Chronic cutaneous lupus erythematosus (CCLE):  Discoid Lupus Erythematosus

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Connective Tissue Diseases Dr. Vinitha Varghese Panicker Associate Professor, Department of Dermatology, Amrita Institute of Medical Sciences & Research Centre, Cochin Digital Lecture Series : Chapter 19 CONTENTS Lupus erythematosus (DLE / SCLE / SLE) Scleroderma (Morphoea / Systemic Sclerosis) Dermatomyositis / Polymyositis Rheumatoid arthritis Sjogrens syndrome Mixed connective tissue disease Antiphospholipid antibody syndrome MCQs Photo Quiz Lupus Erythematosus (LE)- Types Cutaneous lupus erythematosus is classified into three subtypes: Acute cutaneous lupus erythematosus (ACLE): Malar rash, Morbilliform rash, bullous lesions Subacute cutaneous lupus erythematosus (SCLE): Annular, Papulosquamous Chronic cutaneous lupus erythematosus (CCLE): Discoid Lupus Erythematosus Pathogenesis of Cutaneous LE Genetic Factors - HLA susceptibilty Environmental triggers - UV exposure induces cytokine release and apoptosis. Immunologic factors - Malfunction of T regulatory cells (T reg) Role of IL-18 Localised ACLE - Characteristic butterfly facial rash Generalised ACLE - Widespread maculopapular rash in a photo-distributed pattern Acute Cutaneous LE Non - scarring; papulosquamous / annular polycyclic lesions. Vesiculation, crusting, hypopigmentation, telangiectasia, alopecia, photosensitivity, Raynauds phenomenon. Sites : above waist, neck, arms. Systemic involvement 35%. ANA, anti - Ro, anti - La. Subacute Cutaneous LE DLE is a relatively benign disorder of the skin, characterized by well defined, reddish, scaly patches which tend to heal with atrophy, scarring and pigmentary changes. The histology is characteristic. Female : Male - 2 : 1 Onset - second-fourth decade of life Family history : 4% Genetic factors - HLA B7, B8 Chronic: Discoid Lupus Erythematosus (DLE) Discoid rash Histopathology : Epidermal atrophy, basal layer liquefaction, lymphocytic dermal infiltrate and Civatte bodies. Differential diagnosis : Polymorphous Light eruption, Morphoea, Lichen planus, Lupus vulgaris, Sarcoidosis. Diagnosis Definition : A systemic disease with immunopathological abnormalities affecting various organs particularly the skin, joints and vasculature. Females > males Onset: early adult life Systemic LE Fever (52%), lymphadenopathy Arthritis (84%) and arthralgia Cutaneous lesions : specific and non -specific Raynauds phenomenon Renal - nephritis or as nephrotic syndrome. Lung-pleural effusion, alveolitis, interstitial lung disease Cardiac-pericardial effusion, myocardial infarction, Libman-sacks endocarditis Clinical features CNS involvement : migraine, epilepsy, neuropathy. GIT and hepatic involvement : vasculitis of gut, ascites, pancreatitis, autoimmune hepatitis. Splenomegaly, hepatitis, cirrhosis. Hematologic-Anemia, leukopenia, Thrombocytopenia. Ocular - Conjunctvitis, episcleritis, Retinal vasculitis. LE specific ACLE Malar rash SCLE Annular, Psoriasiform CCLE Plaque Lupus panniculitis Hypertrophic Tumid CCLE Chilblain CCLE Mucosal CCLE Cutaneous lesions Malar rashOral ulcer Vascular: Telangiectasia, Purpura, Thrombophlebitis, Raynauds phenomenon, livedo reticularis, erythema multiforme Alopecia: Lupus hair, Alopecia Areata, Scarring Alopecia Mucus membrane lesions Nail changes Calcinosis cutis Bullous lesions Urticaria Pigmentary abnormalities Sclerodactyly and sclerodermatous changes Papulonodular mucinosis Anetoderma Lichen planus Porphyria cutanea tarda LE Non specific lesions Contd SLE in pregnancy Neonatal LE Drug induced LE Childhood SLE Rowells syndrome Special subsets of LE CBC : anemia, leukopenia, thrombocytopenia ESR : raised Urine analysis, BUN, S. Creatinine False positive VDRL & RA factor LE cell test ANA Anti-DNA, anti-Sm, anti-histone, cryoglobulins, serum complement levels DIF Lupus band test Investigations ANA Patterns ANA PATTERNPREDOMINANT ANTIGENDISEASE PERIPHERALnDNASLE HOMOGENOUSnDNA, histonesSLE NUCLEOLARNucleolar RNASSc, SLE CENTROMEREKinetophoreCREST syndrome SPECKLEDVarious RNPs MCTD, SLE, SSc, Sjogrens syndrome Malar rash Discoid rash Photosensitivity Oral ulcers Nonerosive arthritis Serositis : pleurisy or pericarditis Renal disorder : persistent proteinuria (>0.5g/day) or cellular casts Neurological disorders : seizures or psychosis American College of Rheumatology, ACR criteria Contd Haematological disorders - heamolytic anemia or leukopenia (