connective tissue diseases of the skin
Download
Skip this Video
Download Presentation
Connective Tissue Diseases of the Skin

Loading in 2 Seconds...

play fullscreen
1 / 81

Connective Tissue Diseases of the Skin - PowerPoint PPT Presentation


  • 716 Views
  • Uploaded on

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

loader
I am the owner, or an agent authorized to act on behalf of the owner, of the copyrighted work described.
capcha
Download Presentation

PowerPoint Slideshow about ' Connective Tissue Diseases of the Skin' - curt


An Image/Link below is provided (as is) to download presentation

Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author.While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server.


- - - - - - - - - - - - - - - - - - - - - - - - - - E N D - - - - - - - - - - - - - - - - - - - - - - - - - -
Presentation Transcript
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
ad