Connective tissue diseases of the skin
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Connective Tissue Diseases of the Skin. MBChB IV. Outcomes for this lecture. After this lecture the student should be able to: Recognise and describe scleroderma Recognise and describe the different types of scleroderma

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Connective Tissue Diseases of the Skin

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Connective tissue diseases of the skin

Connective Tissue Diseases of the Skin

MBChB IV


Outcomes for this lecture

Outcomes 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 lesions

Connective Tissue Diseases with Skin Lesions

  • Lupus erythematosus

  • Scleroderma

  • Dermatomyositis

  • Rheumatoid arthritis

  • Reiter’s syndrome


Scleroderma

Scleroderma

  • Variants

  • In common: Fibrosis / sclerosis of dermis

    Cutaneous vascular insufficiency

  • Sometimes systemic fibrosis


Variants of scleroderma

Variants of Scleroderma

  • Systemic sclerosis

  • CREST

  • Morphoea

  • Pseudoscleroderma – porphyria

    lipodermatosclerosis


Systemic sclerosis

Systemic Sclerosis

  • Raynaud’s phenomenon

  • Sclerodactyly

  • Diffuse scleroderma

  • Small oral orifice

  • Sharp tip of nose

  • Telangiectasia

  • Calcification

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


Crest

CREST

  • Calcifications (forearms)

  • Raynaud’s phenomenon

  • Esophageal dysmotility

  • Sclerodactyly

  • Telangiectasia

    By definition: No other systemic involvement


Morphoea

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


Variants of morphoea

Variants of Morphoea

  • Usual plaques (one or many)

  • Liniar / segmental

  • Central forehead (en coup de sabre)

  • Hemifacial involvement


Further examination

Further Examination

  • Skin lesions – helps to predict prognosis

  • Signs of arthritis

  • Blood pressure

  • Signs of lung fibrosis

  • Urine microscopy


Special investigations

Special Investigations

  • Serology: ANF, ENAs

    Anti-Scl-70

  • FBC

  • Renal functions

  • Chest X-rays

  • Lung functions

  • Barium swallow

  • Skin biopsy


Management therapeutic

Management: Therapeutic

  • Vasodilators – prazocin

    nifedipine

  • Corticosteroids systemically – early

  • PUVA

  • NSAIDS

  • Cimetidine / PPIs

  • Chloroquine

  • Methotrexate

  • Cyclosporine A


Management referral

Management: Referral

  • Rheumatology

  • Nephrology


Management advice information

Management: Advice / Information

  • Warn that blood pressure control is crucial

  • Minimize cold exposure

  • Soft diet if dysphagia present


Management follow up

Management: 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 le

Discoid LE

  • Most common

  • Extremely chronic and resistant, mutilating

  • Photo areas

  • 5% Systemic involvement

  • Early diagnosis crucial


Discoid le1

Discoid LE

  • Erythema

  • Deep induration / firmness

  • Hyperkeratotic scaling

  • Follicular plugs

  • Atrophy

  • Edge of hyperpigmentation (grey, black)

  • Hair loss (scarring)

  • Cheilitis


Discoid le management

Discoid LE: Management

  • Sun protection

  • Topical steroids (potent)

  • Chloroquine

  • Exclude systemic involvement


Subacute cutaneous le

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

Systemic LE

  • Acute, life threatening disease

  • Photosensitive rash

  • Systemic involvement (criteria)


Systemic lupus erythematosus

Systemic 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 erythematosus1

Systemic Lupus Erythematosus

  • Arthritis

  • Nephropathy

  • Serositis (pleuritis, pericarditis)

  • Hematological: Thrombocytopenia

    Leucopenia

    Anemia

  • Neurological involvement: Epilepsy, stroke

  • Hepatitis


Le serology

LE Serology

  • ANF (90% sensitive, 70% specific)

  • Anti-dsDNA (100% specific, 70% sensitive)

  • Anti-Sm (renal involvement)

  • ENAs (little importance, SCLE)


Other investigations

Other Investigations

  • FBC

  • ESR

  • Urine

  • Renal functions

  • Chest X-rays


Lupus erythematosus treatment

Lupus Erythematosus: Treatment

  • Topical steroids

  • Chloroquine

  • Sun blockers

  • NSAIDS

  • Systemic corticosteroids, cyclophosphamide


Dermatomyositis

Dermatomyositis

  • Skin + muscle + joints (single or combinations)

  • Adult and childhood types

  • Paraneoplastic (16%)

  • Overlap syndromes


Dermatomyositis skin lesions

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

Dermatomyositis: Diagnosis

  • Clinical

  • Serology: Erratic

  • Muscle enzymes (CK, LD, Aldolase)

  • Muscle biopsy

  • Look for underlying neoplasia


Rheumatoid arthritis

Rheumatoid Arthritis

  • Skin lesions: Nodules

    Vasculitis

    Many others


Reiter s syndrome

Reiter’s Syndrome

  • Severe (pustular) psoriasis

  • Keratoderma blenorragica

  • Circinate balanitis

  • Arthritis

  • Urethritis

  • Iridosiclitis


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