Transcript Blank Construction

Connective Tissue Diseases of the
Skin
MBChB IV
Outcomes for this lecture
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After this lecture the student should be able to:
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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 Lesions
 Lupus erythematosus
 Scleroderma
 Dermatomyositis
 Rheumatoid arthritis
 Reiter’s syndrome
Scleroderma
 Variants
 In common: Fibrosis / sclerosis of dermis
Cutaneous vascular insufficiency
 Sometimes systemic fibrosis
Variants of Scleroderma
 Systemic sclerosis
 CREST
 Morphoea
 Pseudoscleroderma – porphyria
lipodermatosclerosis
Systemic Sclerosis
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Raynaud’s phenomenon
Sclerodactyly
Diffuse scleroderma
Small oral orifice
Sharp tip of nose
Telangiectasia
Calcification
Plus: Arthritis, esophageal dysmotility, lung fibrosis, hypertension,
etc
CREST
 Calcifications (forearms)
 Raynaud’s phenomenon
 Esophageal dysmotility
 Sclerodactyly
 Telangiectasia
By definition: No other systemic involvement
Morphoea
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
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Variants of Morphoea
 Usual plaques (one or many)
 Liniar / segmental
 Central forehead (en coup de sabre)
 Hemifacial involvement
Further Examination
 Skin lesions – helps to predict prognosis
 Signs of arthritis
 Blood pressure
 Signs of lung fibrosis
 Urine microscopy
Special Investigations
Serology: ANF, ENAs
Anti-Scl-70
 FBC
 Renal functions
 Chest X-rays
 Lung functions
 Barium swallow
 Skin biopsy
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Management: Therapeutic
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Vasodilators – prazocin
nifedipine
Corticosteroids systemically – early
PUVA
NSAIDS
Cimetidine / PPIs
Chloroquine
Methotrexate
Cyclosporine A
Management: Referral
 Rheumatology
 Nephrology
Management: Advice / Information
 Warn that blood pressure control is crucial
 Minimize cold exposure
 Soft diet if dysphagia present
Management: Follow-up
 Regularly – 3 monthly
 Ask about arthralgia, dysphagia, dyspnea
 Measure blood pressure
 Tests urine
 Lung functions
Lupus Erythematosus
 Chronic (Discoid LE)
 Subacute LE
 Systemic LE
Discoid LE
 Most common
 Extremely chronic and resistant, mutilating
 Photo areas
 5% Systemic involvement
 Early diagnosis crucial
Discoid LE
Erythema
 Deep induration / firmness
 Hyperkeratotic scaling
 Follicular plugs
 Atrophy
 Edge of hyperpigmentation (grey, black)
 Hair loss (scarring)
 Cheilitis
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Discoid LE: Management
 Sun protection
 Topical steroids (potent)
 Chloroquine
 Exclude systemic involvement
Subacute 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 LE
 Acute, life threatening disease
 Photosensitive rash
 Systemic involvement (criteria)
Systemic Lupus Erythematosus
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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 Erythematosus
 Arthritis
 Nephropathy
 Serositis (pleuritis, pericarditis)
 Hematological: Thrombocytopenia
Leucopenia
Anemia
 Neurological involvement: Epilepsy, stroke
 Hepatitis
LE Serology
 ANF (90% sensitive, 70% specific)
 Anti-dsDNA (100% specific, 70% sensitive)
 Anti-Sm (renal involvement)
 ENAs (little importance, SCLE)
Other Investigations
 FBC
 ESR
 Urine
 Renal functions
 Chest X-rays
Lupus Erythematosus: Treatment
 Topical steroids
 Chloroquine
 Sun blockers
 NSAIDS
 Systemic corticosteroids, cyclophosphamide
Dermatomyositis
 Skin + muscle + joints (single or combinations)
 Adult and childhood types
 Paraneoplastic (16%)
 Overlap syndromes
Dermatomyositis: 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: Diagnosis
 Clinical
 Serology: Erratic
 Muscle enzymes (CK, LD, Aldolase)
 Muscle biopsy
 Look for underlying neoplasia
Rheumatoid Arthritis
 Skin lesions: Nodules
Vasculitis
Many others
Reiter’s Syndrome
 Severe (pustular) psoriasis
 Keratoderma blenorragica
 Circinate balanitis
 Arthritis
 Urethritis
 Iridosiclitis