Connective Tissue Diseases of the Skin

Connective Tissue Diseases of the Skin

MBChB IV

Outcomes for this lectureOutcomes for this lecture After this lecture the student should be able to: Recognise and describe scleroderma Recognise and describe the different types of scleroderma Recognise and describe the symptoms and signs of systemic sclerosis (cutaneous and systemic) Name and apply the investigations that has to be done in cases of scleroderma (clinical, side room tests,

imaging and laboratory tests) Broadly name the management of scleroderma Recognise and describe in detail the clinical presentation of chronic discoid lupus erythematosus (DLE) Broadly describe the management of DLE Be aware of the condition named subacute cutaneous lupus erythematosus Recognise and describe in detail the skin signs of acute systemic lupus erythematosus (SLE) Name the systemic involvement of SLE and recognise and describe the symptoms and signs of systemic

involvement Name the serological tests that may be done to further investigate SLE and discuss the implications thereof Name the special investigations (imaging and laboratory tests) that have to be done to investigate SLE Describe in broad terms the management of SLE Recognise and describe the clinical presentation of dermatomyositis (skin and muscle) Recognise and describe the clinical presentation of cutaneous vasculitis and rheumatoid nodules

Connective Tissue Diseases with Skin LesionsConnective Tissue Diseases with Skin Lesions

Lupus erythematosus Scleroderma Dermatomyositis Rheumatoid arthritis Reiter’s syndrome

SclerodermaScleroderma Variants In common: Fibrosis / sclerosis of dermis Cutaneous vascular insufficiency Sometimes systemic fibrosis

Variants of SclerodermaVariants of Scleroderma Systemic sclerosis CREST Morphoea Pseudoscleroderma – porphyria lipodermatosclerosis

Systemic SclerosisSystemic Sclerosis Raynaud’s phenomenon Sclerodactyly Diffuse scleroderma Small oral orifice Sharp tip of nose Telangiectasia Calcification

Plus: Arthritis, esophageal dysmotility, lung fibrosis, hypertension, etc

CRESTCREST Calcifications (forearms) Raynaud’s phenomenon Esophageal dysmotility Sclerodactyly Telangiectasia

By definition: No other systemic involvement

MorphoeaMorphoea Localised plaques of scleroderma Seldom systemic involvement Begins as pink-purple macule Later central induration / hardening Violaceus edge persists Epidermis sometimes shiny, atrophic, depigmented,

spotty Sometimes deep atrophy

Variants of MorphoeaVariants of Morphoea Usual plaques (one or many) Liniar / segmental Central forehead (en coup de sabre) Hemifacial involvement

Further ExaminationFurther Examination Skin lesions – helps to predict prognosis Signs of arthritis Blood pressure Signs of lung fibrosis Urine microscopy

Special InvestigationsSpecial Investigations Serology: ANF, ENAs Anti-Scl-70 FBC Renal functions Chest X-rays Lung functions Barium swallow Skin biopsy

Management: TherapeuticManagement: Therapeutic Vasodilators – prazocin nifedipine Corticosteroids systemically – early PUVA NSAIDS Cimetidine / PPIs Chloroquine Methotrexate Cyclosporine A

Management: ReferralManagement: Referral Rheumatology Nephrology

Management: Advice / InformationManagement: Advice / Information Warn that blood pressure control is crucial Minimize cold exposure Soft diet if dysphagia present

Management: Follow-upManagement: Follow-up Regularly – 3 monthly Ask about arthralgia, dysphagia, dyspnea Measure blood pressure Tests urine Lung functions

Lupus Erythematosus Lupus Erythematosus Chronic (Discoid LE) Subacute LE Systemic LE

Discoid LEDiscoid LE Most common Extremely chronic and resistant, mutilating Photo areas 5% Systemic involvement Early diagnosis crucial

Discoid LEDiscoid LE Erythema Deep induration / firmness Hyperkeratotic scaling Follicular plugs Atrophy Edge of hyperpigmentation (grey, black) Hair loss (scarring) Cheilitis

Discoid LE: ManagementDiscoid LE: Management Sun protection Topical steroids (potent) Chloroquine Exclude systemic involvement

Subacute Cutaneous LESubacute Cutaneous LE Photosensitive disease Face, shoulders, back, arms Annular / psoriasiforme Little / no atrophy ENAs positive (anti-Ro, anti-La) Arthritis Brain -, lung involvement, not nephropathy

Systemic LESystemic LE Acute, life threatening disease Photosensitive rash Systemic involvement (criteria)

Systemic Lupus ErythematosusSystemic Lupus Erythematosus Skin lesions: Malar erythema (butterfly) Epidermal necrosis (wide spread) Discoid lesions Telangiectasia Vasculitis (hands, fingers) Chronic, recurrent oral ulcers Diffuse alopecia (non-scarring) Photosensitivity

Systemic Lupus ErythematosusSystemic Lupus Erythematosus

Arthritis Nephropathy Serositis (pleuritis, pericarditis) Hematological: Thrombocytopenia Leucopenia Anemia Neurological involvement: Epilepsy, stroke Hepatitis

LE SerologyLE Serology ANF (90% sensitive, 70% specific) Anti-dsDNA (100% specific, 70% sensitive) Anti-Sm (renal involvement) ENAs (little importance, SCLE)

Other InvestigationsOther Investigations FBC ESR Urine Renal functions Chest X-rays

Lupus Erythematosus: TreatmentLupus Erythematosus: Treatment Topical steroids Chloroquine Sun blockers NSAIDS Systemic corticosteroids, cyclophosphamide

DermatomyositisDermatomyositis Skin + muscle + joints (single or combinations) Adult and childhood types Paraneoplastic (16%) Overlap syndromes

Dermatomyositis: Skin LesionsDermatomyositis: Skin Lesions Heliotropic eyelids Eye lid edema Red face, LE-like Psoriasiforme arms Gottron-papules knuckles Nail fold erythema / telangiectasia / scars Deep, tumoral calcification

Dermatomyositis: DiagnosisDermatomyositis: Diagnosis Clinical Serology: Erratic Muscle enzymes (CK, LD, Aldolase) Muscle biopsy Look for underlying neoplasia

Rheumatoid ArthritisRheumatoid Arthritis Skin lesions: Nodules Vasculitis Many others

Reiter’s SyndromeReiter’s Syndrome Severe (pustular) psoriasis Keratoderma blenorragica Circinate balanitis Arthritis Urethritis Iridosiclitis