cutaneous manifestations of systemic diseases hayden h. franks, md june 13, 2013
TRANSCRIPT
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Cutaneous Manifestations of
Systemic DiseasesHayden H. Franks, MD
June 13, 2013
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Who Is This Guy?
Private practice Dermatologist
Clinics in Little Rock and Texarkana
Fellow of the American Academy of Dermatology
Diplomate of the American Board of Dermatology
Assistant Clinical Professor of Dermatology, UAMS AHEC SW
Honorary Member of the Arkansas Academy of Family Physicians
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Disease Categories
Autoimmune Diseases
Endocrine Diseases
Cardiopulmonary Diseases
Gastrointestinal Diseases
Neurological Diseases
Diseases not Otherwise Specifiied
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Cutaneous Manifestations of Systemic Diseases
Frequently encountered
May be the initial sign of internal disease
May occur late in the course of the disease
May assist in making the diagnosis
May be obvious or subtle
Overlap of Family Practice and Dermatology
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AUTOIMMUNE DISEASES
Systemic Lupus Erythematosus
Scleroderma
Dermatomyositis
Rheumatoid Arthritis
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Systemic Lupus Erythematosus
Autoimmune, systemic disease affecting multiple organ systems
The most common connective tissue disease
Especially prevalent in black women – Prevalence 1/250
Cutaneous lesions present in 85% of patients
Of the 11 Classic Criteria for diagnosing SLE, 4 involve the skin or mucus membranes
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Systemic Lupus Erythematosus
Malar (Butterfly) rash is the “classic presentation”
May be distinct or subtle
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Systemic Lupus Erythematosus
Fixed erythema, flat or raised, over malar eminences
Spares the Nasolabial Folds
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Systemic Lupus Erythematosus
Discoid Rash is “classic” as well
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Systemic Lupus Erythematosus
Erythematous, patches and plaques, with adherent scales, follicular plugging and atrophic scarring
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Systemic Lupus Erythematosus
Photosensitivity – rash as an unusual reaction to sunlight
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Systemic Lupus Erythematosus
Oral Ulcers – usually painless and may be nasopharyngeal
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Systemic Lupus Erythematosus
Presentation isn’t always “classic”
High index of suspicion
Alopecia and rash may be anywhere on skin
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Systemic Lupus Erythematosus
Diagnosis is based on presence of multisystem disease and presence of antinuclear antibodies
Treatment is multifactorial with corticosteroids being the mainstay still
Sunscreen
Antimalarials, methotrexate, dapsone and biologics now are commonly used
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Scleroderma
Chronic autoimmune disease of unknown cause that affects the microvasculature and loose connective tissue
Characterized by fibrosis and obliteration of vessels in skin, lungs, GI tract, kidneys and heart
May be localized (Morphea) or systemic (Systemic Scleroderma)
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Scleroderma (Morphea)
Morphea – benign and self limited
Usually single or few in number
Red, then white, atrophic, indurated with alopecia
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Scleroderma (Morphea)
Treatment is unsatisfactory
Topical or intralesional steroids, PUVA
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Systemic Scleroderma (SSc)
Four times more common in women
10 year survival rate of 21-71%
Clinical manifestations depend on the sites involved
Initial complaints are usually Raynaud’s phenomenon or chronic, non pitting edema of hands and fingers or migratory polyarthritis
Disease may extend to involve upper extremities, trunk, face and finally the lower extremities
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Systemic Scleroderma (SSc)
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Systemic Scleroderma (SSc)
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Systemic Scleroderma (SSc)
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Systemic Scleroderma (SSc) Diagnosis
Autoantibodies to Fibrillin 1, Rheumatoid Factor, Anti SS DNA, Anti RNA Polymerase 3, Antitopoisomerase 1, Anticentromere Antibodies
Skin Biopsy
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Systemic Scleroderma (SSc) Treatment
Treatment is unsatisfactory
Immunosuppressive Drugs of numerous types
Methotrexate, Cyclosporine, Imuran
Biologics
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Dermatomyositis
The most common idiopathic inflammatory myopathy
May occur at any age
Unknown etiology
Autoimmune Disease
Progressive weakness of trunk and major limb muscles
Difficulty in rising from a chair or climbing stairs
Impaired mobility and some muscle tenderness
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Dermatomyositis
Bilateral muscle weakness that is progressive
Skin lesions are almost always present from the onset
Maculopapular erythema over bony prominences such as the knuckles, elbows and knees
Red to violaceous plaques with telangiectasias and scales
Gottron’s Papules – polymorphic, erythematous and atrophic plaques
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Dermatomyositis
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Dermatomyositis
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Dermatomyositis
Heliotrope Rash – Periorbital erythema
Nail Margin Telangiectasias
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Dermatomyositis Diagnosis
Elevated serum muscle enzymes (CK) and Aldolase
Antinuclear Antibodies
Muscle biopsy – segmental muscle fiber fibrosis, interstitial inflammation and vasculopathy
Skin biopsy – Focal vacuolar degeneration of basal cells, basement membrane degeneration and epidermal atrophy
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Dermatomyositis Treatment
Primary treatment remains Prednisone 1mg/kg/day
Plasmapheresis
Cyclosporine
Dapsone
?Biologics
Physical Therapy
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Rheumatoid Arthritis
Disease affects up to 2% of adult women
Onset is sudden or insidious
Symmetric polyarthritis that affects the proximal interphalangeal and metacarpophalangeal joints, the wrists, ankles, knees and cervical spine
Stiffness, painful, warm and tender joints
Fever, weight loss and anemia are prominent
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Rheumatoid Arthritis
Rheumatoid Nodules – discrete, non tender subcutaneous tumors
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Rheumatoid Arthritis
Vascular Lesions – erythema of palms and digital infarcts
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Rheumatoid Arthritis
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Rheumatoid Arthritis
Gravitational ulcers – most common
Arteritic ulcers – actually rare until advanced disease
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Rheumatoid Arthritis
Laboratory Workup – Rheumatoid Factor and ANA
Treatment – Prednisone, Methotrexate, Biologics
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ENDOCRINE DISEASES
Diabetes Mellitus
Thyroid Disease
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Diabetes Mellitus
The skin shares both in the effects of acute metabolic derangements and in the chronic degenerative complications of diabetes.
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Diabetes Mellitus
Infection
Diabetic Dermopathy
Thickened skin, stiff joints and Scleredema Adultorum
Necrobiosis Lipoidica Diabeticorum
Vitiligo
Acanthosis Nigricans
Kyrle’s Disease (Reactive Perforating Collagenosis)
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Diabetes Mellitus
Bacterial and fungal infections
Furunculosis, Cellulitis, Erythrasma, and Candidiasis
Hyperglycemia leads to abnormalities in leukocyte function including diminished chemotaxis and phagocytosis
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Diabetes Mellitus
Bacterial Infections
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Diabetes Mellitus
Cellulitis
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Diabetes Mellitus
Erythrasma
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Diabetes Mellitus
Candidiasis
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Diabetes Mellitus
Diabetic Dermopathy – atrophic, circumscribed brownish lesions usually on the lower extremities
They resemble post traumatic scarring
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Diabetes Mellitus
Thickened Skin, Stiff Joints and Scleredema Adultorum
33% of Diabetics have tight, indurated and waxy skin over the dorsa of the hands
Scleredema Adultorum is strongly correlated with IDDM
Consists of induration of the skin beginning on the posterior and lateral aspect of the neck, is painless and may be progressive
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Diabetes Mellitus
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Diabetes Mellitus
Scleredema Adultorum
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Diabetes Mellitus
Necrobiosis Lipoidica Diabeticorum
Occurs in 0.3% of IDDM Patients
Very distinct
Asymptomatic, atrophic, yellow to brown patches classically on the lower extremities
Telangiectasias are prominent
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Diabetes Mellitus
Necrobiosis Lipoidica Diabeticorum
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Diabetes Mellitus
Vitiligo
Many times is associated with IDDM, Thyroid Disease and Systemic Lupus Erythematosus
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Diabetes Mellitus
Acanthosis Nigricans – characterized as velvety, papillomatous hyperplasia of the epidermis with intense hyperpigmentation
Axillary, inguinal and inframammary folds and the neck
Found in association with several endocrinopathies – including Cushing’s Disease, Polycystic Ovary Disease and IDDM
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Diabetes Mellitus
Acanthosis Nigricans
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Diabetes Mellitus
Kyrle’s Disease – rare and characterized by hyperkeratotic, follicular and perifollicular papules
Transepidermal elimination of altered collagen
Also strongly associated with renal disease
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Diabetes Mellitus
Kyrle’s Disease
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Thyroid Disease
Thyroid hormones have diverse primary sites of action at the level of the cell membrane, mitochondria and gene transcription that regulate functional properties and metabolism of most cells of the body including the keratinocytes and fibroblasts of the skin.
Thyroid hormones affect production of collagen and mucopolysacccharides by dermal fibroblasts.
Lack of thyroid hormone affects all of the above
Excess thyroid hormone does not
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Thyroid Disease
Thyrotoxicosis (Hyperthyroidism) – due to Grave’s Disease or Toxic Nodular Goiter
Skin is warm, moist, flushed and excess sweating
Alopecia
Uncommonly pruritus, vitiligo
Pretibial Myxedema
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Thyroid Disease
Pretibial Myxedema – an infiltrative dermopathy, usually over anterior tibia and dorsa of feet.
Bilateral, pink, violacous or flesh colored confluent papules
Diffuse brawny edema
Correction of thyrotoxicosis has no effect on the skin lesions
Half of cases occur after patient has been rendered euthyroid
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Thyroid Disease
Pretibial Myxedema
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Thyroid Disease (Hypothyroidism)
Skin is cold, xerotic and pale
Vasoconstriction
Epidermis is thin, hyperkeratotic
Fine wrinkling resembles parchment paper
Yellow discoloration especially of palms, soles and nasolabial folds
Hair is dry, coarse, brittle and grows slowly
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Thyroid Disease (Hypothyroidism)
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Thyroid Disease (Hypothyroidism)
Myxedema – dermal accumulation of mucopolysaccharides (hyaluronic acid and chondroitin sulfate)
Usually located acrally
May be diffuse or focal (papules)
Broad nose, thick lips, large smooth tongue
Drooping eyelids and an expressionless face
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Thyroid Disease (Hypothyroidism)
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CARDIOPULMONARY DISEASES
Coronary Heart Disease
Subacute Bacterial Endocarditis
COPD
Cystic Fibrosis
Asthma
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Coronary Heart Disease
Familial Hyperlipidemia – a group of metabolic disorders with elevated plasma cholesterol and or triglyceride levels. Often see Xanthomatosis on the skin
Earlobe Crease – there is an association between CAD and a diagonally positioned skin crease along the earlobe that may be unilateral or bilateral.
Post Bypass Skin changes
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Coronary Heart Disease
Xanthomatosis
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Coronary Heart Disease
Earlobe Crease
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Coronary Heart Disease
Postbypass Skin Changes –
Saphenous Vein Graft Site Dermatitis
Tinea Pedis
Stasis Edema and Stasis Dermatitis
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Coronary Heart Disease
Saphenous Vein Graft Site Dermatitis
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Coronary Heart Disease
Tinea Pedis
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Coronary Heart Disease
Stasis Edema and Stasis Dermatitis
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Coronary Heart Disease
Stasis Edema and Stasis Dermatitis
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Coronary Heart Disease
Stasis Dermatitis vs Cellulitis
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Actinic Purpura
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Subacute Bacterial Endocarditis
Petechiae are the most common mucocutaneous manifestation of bacterial endocarditis – small red or violaceous macules that don’t blanch – not associated with platelet dysfunction Osler’s Nodes Janeway Lesions Subungual Splinter Hemorrhages Cutaneous Purpura and Petechiae Conjunctival Petechiae (Roth’s Spots)
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Subacute Bacterial Endocarditis
Petechiae
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Subacute Bacterial Endocarditis
Osler’s Nodes – painful hemorrhagic macules and papules located on digital tufts
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Subacute Bacterial Endocaridits
Janeway Lesions- Nontender hemorrhagic macules and papules located on palms and soles
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Subacute Bacterial Endocarditis
Subungual Splinter Hemorrhages
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Subacute Bacterial Endocarditis
Conjunctival Petechiae (Roth’s Spots)
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Chronic Obstructive Pulmonary Disease (COPD)
Actually a group of disorders including chronic bronchitis, bronchiectasis, emphysema and asthma
Incidence is increasing and actually approaching that of cardiac disease
Environmental and genetic influences
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Chronic Obstructive Pulmonary Disease (COPD)
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Chronic Obstructive Pulmonary Disease (COPD)
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Cystic Fibrosis
Autosomal Recessive disorder of the exocrine glands that subsequently involves the tracheobronchial tree, pancreas and GI tract
Cutaneous features result from increased amounts of electrolyte in the sweat that leads to excess skin wrinkling of palms and soles when immersed in water.
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Cystic Fibrosis
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Asthma
Asthma – Eczema Complex (Atopy) Association of asthma, atopic eczema and
allergic rhinitis are well documented Mediators of this inflammatory response may
be released by sensitized IgE – Mast cell complexes
Dust, pollen, dander, heat, dry conditions, exercise and other allergens all may trigger an outbreak
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Asthma
Asthma – Atopic Dermatitis (Atopy)
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Asthma
Asthma – Atopic Dermatitis (Atopy)
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GASTROINTESTINAL DISEASES
Inflammatory Bowel Disease
Celiac Disease
Hepatitis
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Cutaneous Manifestations of Gastrointestinal Diseases
Jaundice
Melanosis
Nail Changes
Edema
Purpura
Pruritus
Vascular Changes Spider
Telangiectasias Palmar Erythema
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Inflammatory Bowel Disease
Inflammatory Bowel Disease – Ulcerative Colitis and Crohn’s Disease
Skin complications are similar in these two diseases
Pyoderma Gangrenosum Erythema Nodosum Aphthous Ulcers Lichen Planus
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Pyoderma Gangrenosum
Pyoderma Gangrenosum Rare, destructive, inflammatory skin disease Progressively enlarging ulcers with raised,
tender, undermined borders Most commonly seen on legs but may be
anywhere May be solitary or multiple May be isolated or seen with Inflammatory
Bowel Disease, Polyarthritis or certain malignancies
Affects 5 to 10% of Inflammatory Bowel Disease patients
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Pyoderma Gangrenosum
Pyoderma Gangrenosum
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Pyoderma Gangrenosum
Pyoderma Gangrenosum
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Erythema Nodosum
Erythema Nodosum Cutaneous reaction pattern consisting of
inflammatory, spontaneously regressing, tender, nodular lesions located primarily over the extensor surfaces of the lower legs
Septal panniculitis without vasculitis Is associated with a wide variety of disease
processes Immunologic pathogenesis In addition to occurring in Crohn’s and UC,
also seen with infections, Sarcoidosis and drugs (Sulfonamides and Oral Contraceptives )
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Erythema Nodosum
Erythema Nodosum
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Erythema Nodosum
Erythema Nodosum
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Aphthous Ulcers
Aphthous Ulcers Small, shallow, well circumscribed ulcers Oral mucosa Appear suddenly and are painful Resolve within 2 weeks only to recur May be related to stress or menses Very common – may affect up to 20% of
general population
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Aphthous Ulcers
Aphthous Ulcers
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Aphthous Ulcers
Aphthous Ulcers
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Lichen Planus
Lichen Planus Skin eruption consisting of violaceous,
scaling, angular papules and plaques Flexor surfaces and mucus membranes are
classic locations Symmetrically distributed Usually pruritic Incidence of around 0.5% Usually isolated but may be associated with
underlying disease (UC and Crohn’s Disease)
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Lichen Planus
Lichen Planus
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Lichen Planus
Lichen Planus
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Celiac Disease
Celiac Disease – also known as Celiac Sprue. autoimmune disease of the small intestine Abdominal pain, discomfort, diarrhea,
constipation, failure to thrive (children) May lead to vitamin deficiencies due to
malabsorption Increasing in incidence due to improved
screening Associated with Dermatitis Herpetiformis
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Dermatitis Herpetiformis
Dermatitis Herpetiformis Intensely pruritic, chronic, papulovesicular
eruption distributed symmetrically on extensor surfaces classically over the elbow
Most patients have an associated gluten sensitive enteropathy that may be asymptomatic
Universally responsive to Dapsone Most patients with DH have Celiac Disease
although many are mild or asymptomatic Gluten free diet is beneficial
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Dermatitis Herpetiformis
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Dermatitis Herpetiformis
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Cutaneous Manifestations of Hepatitis
Urticaria
Vasculitis
Polyarteritis Nodosa
Relapsing Papulovesicular Rash
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Urticaria in Hepatitis
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Vasculitis in Hepatitis
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NEUROLOGICAL DISEASES
Parkinson’s Disease
Cerebrovascular Accident (Stroke)
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Parkinson’s Disease
Degenerative disorder of the Central Nervous System
Motor symptoms result from death of Substantia Nigra cells of the Midbrain which generate Dopamine
Tremor at rest, bradykinesia, rigidity, postural instability
Later in disease dementia and neuropsychiatric problems may occur
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Parkinson’s Disease
Hyperhidrosis especially of the face and palms Peripheral sweat gland function is controlled
by sympathetic nervous system which is altered in PD patients
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Parkinson’s Disease
Seborrheic Dermatitis Overexcretion of sebum on the face which is
thought to be caused by hyperactivity of the Parasympathetic component of the Autonomic Nervous System
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Parkinson’s Disease
Seborrheic Dermatitis
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Cerebrovascular Accident (Stroke)
Changes are due to Unilateral disturbance in autonomic function -
including skin temperature, turgor, xerosis and sweating
Loss of motor function –including edema and ulceration (pressure ulcers)
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Cerebrovascular Accident (Stroke)
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Cerebrovascular Accident (Stroke)
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DISEASES NOT OTHERWISE SPECIFIED
Sarcoidosis
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Sarcoidosis
A granulomatous disease most commonly associated with lesions of the lungs and bilateral hilar lymphadenopathy
Multisystem disease that can present in many ways
1/3 of pateints complain of fatigue, fever and weight loss
1/3 of patients have dyspnea, cough and chest pain
Serum ACE levels raised in 60% of patients
Skin lesions occur in 40 % of patients
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Sarcoidosis
Skin Lesions Lupus Pernio Skin Plaques Subcutaneous nodules Erythema Nodosum
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Sarcoidosis
Lupus Pernio – the most characteristic of all Sarcoid skin lesions Chronic, violaceous, indurated papules and
plaques with a predilection for the face especially the nose
May be associated with advance pulmonary disease
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Sarcoidosis
Lupus Pernio
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Sarcoidosis
Lupus Pernio
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Sarcoidosis
Erythema Nodosum