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Skin and Systemic disease

Skin and Systemic disease. Skin and Systemic disease. Systemic diseases with Cutaneous manifestations: Skin mirrors many internal diseases which are often first noticed due to cutaneous manifestations. Skin involvement is an integral part of many systemic illnesses.

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Skin and Systemic disease

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  1. Skin and Systemic disease

  2. Skin and Systemic disease • Systemic diseases with Cutaneous manifestations: Skin mirrors many internal diseases which are often first noticed due to cutaneous manifestations. Skin involvement is an integral part of many systemic illnesses. Examples: Systemic Lupus Erythematosus, Systemic Sclerosis, Xanthomas, Sarcoidosis, Paraneoplastic syndromes • Cutaneous diseases with Systemic involvement: Systemic involvement may be a feature of certain skin diseases. Examples: Erythroderma, Lepra reactions, Drug reactions

  3. Skin and Systemic disease • Erythroderma • Endocrine Diabetes Mellitus Thyroid disorders Others: Parathyroid, Adrenal, Gonadal dysfunction • Renal disorders • Gastro-intestinal and Liver disorders • Internal malignancy: Paraneoplastic • Other Systemic disorders: Sarcoidosis Xanthomas Porphyrias

  4. Erythroderma (Exfoliative dermatitis) • Definition : Erythema and scaling involving 90% or more of the skin surface area

  5. Erythroderma : Etiology • Pre - existing skin disease: Psoriasis, Atopic dermatitis, Stasis dermatitis, Contact dermatitis, Seborrhoeic dermatitis, Ichthyosis, Pemphigusfoliaceous, Extensive dermatophytosis, Norwegian scabies • Systemic disease: Internal malignancy especially mycosis fungoides and Sezary syndrome • Drugs Penicillins, Sulphonamides, Chloroquine, Sulphonylureas • Idiopathic

  6. Erythroderma: Clinical features • Erythema • Scaling • Alopecia • Nail dystrophy and shedding • Lymphadenopathy

  7. Erythroderma: Complications • Dehydration • Hypoalbuminemia • Loss of muscle mass • Hypothermia / Hyperthermia • Tachycardia and high- output cardiac failure • Oedema

  8. Erythroderma: Management • Clinical assessment and biochemistry to see if metabolic derangement present • Admit if needed, close monitoring of biochemical parameters • Maintain electrolyte balance • Replace protein and iron • Treat cause if known viz. acitretin or methotrexate in psoriasis • If drug induced or idiopathic, oral prednisolone at 1mg/kg body weight to be tapered slowly

  9. Endocrine system • There are many cutaneous manifestations of various endocrine disorders • Cutaneous manifestations of diabetes mellitus and thyroid disorders are most often encountered by the dermatologist

  10. Diabetes Mellitus: Cutaneous manifestations • Cutaneous Infections: Pyodermas (Carbuncle), Candida, Pseudomonas, Dermatophytosis • Dermal Manifestations: Diabetic thick skin, waxy skin • Vascular Manifestations: Macroangiopathy, diabetic dermopathy, pigmented purpura, periungualtelangiectasia, erysipelas like erythema • Other Skin Markers: Yellow nails, diabetic bullae, granulomaannulare, necrobiosislipoidica, lichen planus, fat hypertrophy

  11. Diabetes Mellitus: Cutaneous manifestations

  12. Diabetes Mellitus: Cutaneous manifestations

  13. Hypothyroidism: Cutaneous manifestations • Skin: dry, cool and pale yellow (due to carotenemia) • Hairs: dull, coarse and brittle; diffuse hair loss and loss of lateral third of eyebrows • Nails: thin, striated and brittle. • Myxedema: Skin of face and acral areas is thick and boggy (non-pitting edema)

  14. Hyperthyroidism: Cutaneous manifestations • Skin: flushed, moist, smooth, velvety to touch • Hair: thin and friable; diffuse alopecia • Nails: soft, friable, koilonychia, onycholysis • Graves’ disease: Pretibialmyxedema (waxy papules or plaques with ‘peau de orange’ look) may extend to hands, feet, thighs; Thyroid acropachy (drumstick clubbing)

  15. Parathyroid Disorders: Cutaneous manifestations • Hypoparathyroidism: Skin : dry, scaly, eczematous eruptions or exfoliative dermatitis Hair : thin, fragile, patchy alopecia Nails : atrophic, brittle with horizontal ridging • Hyperparathyroidism: Disabling pruritus in primary and secondary hyperparathyroidism (due to chronic renal failure) May be relieved by parathyroidectomy

  16. Disorders of adrenal glands: Cutaneous manifestations • Cushing’s Syndrome: • Skin: thin, fragile with purpura and striae. • Redistribution of body fat: Trunkal obesity, moon facies, buffalo hump and thin limbs • Acne, hirsutism and acanthosisnigricans

  17. Disorders of adrenal glands:Cutaneous Manifestations • Addison’s Disease: Generalised, diffuse brown-black pigmentation of skin and mucosae • Accentuation of pigmentation on: • exposed areas (face, hands, forearms), • flexures (axillae, groins), • bony prominences (knuckles, knees, elbows), • normally pigmented areas (palmar creases, nipples, genitalia) • pre- existing melanocytic nevi • frictional areas (e.g. beltline) • Mucosae (blue black color especially over oral mucosa)

  18. Disorders of sex hormones: Cutaneous manifestations • Excess: • Polycystic ovary syndrome, ovarian tumours, congenital adrenal hyperplasia, Cushing’s disease, prolactinoma, drugs like androgens, anabolic steroids or progestagens • Defeminising and virilising syndromes: • Hirsutism and male pattern alopecia • Thick, oily, hyperhidrotic skin; acne, acanthosisnigricans Contd…

  19. Disorders of sex hormones: Cutaneous manifestations • Deficiency • Hypogonadism (Pituitary or non-pituitary) • Features of hypopituitarism • Absent or sparse axillary and pubic hair in males or female type body hair distribution in males

  20. Renal diseases Renal failure: • Persistent generalisedpruritus, dry, scaly skin • Tendency to develop purpura/ecchymoses on minor trauma • Half and half nails in chronic renal failure show brown red discoloration of their distal half • Pale yellow skin: associated anemia and pitting edema due to accumulation of urochrome or carotene pigments • Uremic frost: deposition of urea crystals on the nose and malar area due to high urea levels • Calcinosis cutis, pseudoporphyriacutaneatarda, nephrogenicfibrosingdermatopathy

  21. Renocutaneous diseases • Systemic Lupus Erythematosus: Discoid lesions, butterfly erythema, palatal ulcer, alopecia, photosensitivity • Systemic Sclerosis: Diffuse skin sclerosis, Raynaud’s phenomenon, telangiectasia, pigmentation and calcinosis • Vasculitides (HenochSchonlein, Wegener’s, Polyarteritisnodosa): Palpable purpura, vesicles, skin infarcts, ulcers • Lepromatous Leprosy: Shiny papulonodules and diffuse infiltration of skin

  22. Gastrointestinal disorders Dysphagia: • Rashes that may extend to esophagus • Infections • Congenital and acquired blistering diseases • Lichen planus • Behcet’s disease • Stevens Johnson Syndrome • Dermatomyositis

  23. Gastrointestinal disorders Bleeding: • Hereditary haemorrhagictelangiectasia • Blue rubber bleb nevi • Ehlers Danlos syndrome • PseudoxanthomaElasticum • Kaposi’s sarcoma

  24. Gastrointestinal disorders Abdominal pain: • Herpes zoster • Angioedema • Porphyria • Anderson – Fabry disease • Vasculitis: HenochSchonleinpurpura, Collagen vascular diseases • Polyposis: Gardner’s syndrome, Peutz-Jeghers syndrome, ulcerative colitis, neurofibromatosis • Ulcerative Colitis and Crohn’s disease • Pancreatitis

  25. Gastrointestinal disorders Inflammatory Bowel Diseases: • Pyodermagangrenosum • Aphthous ulcers • ErythemaNodosum • Malnutrition • Rashes at ileostomy and colostomy sites • Metastatic cutaneousCrohn’s disease

  26. Liver disease: Cutaneous manifestations • Pruritus • Icterus • Pigmentary changes • Spider angiomas • Palmarerythema • Dilated abdominal wall veins • Purpura • Loss of body hair • Gynaecomastia • Peripheral oedema

  27. Paraneoplastic syndromes Internal malignancies: Cutaneous Signs • Acanthosisnigricans • Exfoliative dermatitis • Dermatomyositis • Paraneoplasticpemphigus • Leser-Trélat Sign: Sudden onset multiple seborrhoeickeratoses • Trousseau’s sign: Migratory thrombophlebitis • Sister Mary Joseph nodule • Paget’s disease : underlying adnexal or breast carcinoma

  28. Acanthosisnigricans Causes: • Obesity • Hyperinsulinemia and diabetes • Drugs: Corticosteroids, nicotinic acid, diethylstilbestrol, isoniazid • Adenocarcinomas (Gastro-intestinal) • Malignant acanthosisnigricans has a sudden onset and more extensive distribution: face and dorsal and palmar surfaces of the hands

  29. Sarcoidosis • Non-caseatingepithelioid cell granulomas • 3rd to 4th decade of life • Etiology unknown; possibly infections, genetic, environmental factors • Skin lesions may be papules, nodules, plaques, psoriasiform • Area of involvement ranges from localized to generalized (erythrodermic)

  30. Sarcoidosis Diagnosis: • Skin biopsy is confirmatory • Screening of other systems to rule out systemic involvement • Raised angiotensin converting enzyme levels: systemic involvement Treatment: • Systemic: Steroids, immunosuppressants • Localized: Intralesional or topical steroids

  31. Xanthomas • Sign of systemic metabolic abnormality or a local cellular dysfunction or first sign of hyperlipoproteinemias Clinical Types: • Eruptive, plane, tendinous, tuberous • Xanthelasmapalpebraerum, the commonest type rarely associated with hyperlipidemia Treatment: • Xanthelasma: Chemical agents-trichloroacetic acid, cryosurgery with liquid nitrogen, surgical excision, lasers: CO2 , pulsed dye, Erbium:YAG • Surgical excision for tendinousxanthoma

  32. Porphyrias • Inherited or acquired disease state where intermediate metabolites of haemoglobin synthesis (porphyrinogens) are increased Classification • Erythropoietic • Congenital erythropoietic • Erythropoieticprotoporphyria • Erythropoieticcoproporphyria • Hepatic • Acute intermittent porphyria • ALA dehydratase deficiency • Hereditary coproporphyria • Variegate porphyria • Porphyriacutaneatarda • Hepatoerythrocyticporphyria

  33. Porphyriacutaneatarda • Autosomal dominantly inherited (before age 20) or sporadic non-familial • Acute or chronic exposure to polyhalogenated hydrocarbons-hexachlorobenzene, hepatotoxins or digoxin • Liver disease: Alcoholism, hepatitis C virus, hepatocellular carcinoma • Lupus erythematosus, diabetes mellitus and HIV may be associated

  34. Porphyriacutaneatarda • Photosensitivity: bullae on sun-exposed parts which rupture, erosions heal with scarring and milia formation • Hypertrichosis of cheeks, temples • Sclerodermatous change of the back of neck, pre-auricular area, thorax, fingers and scalp (associated with alopecia) • Pink/coral red fluorescence of urine- Wood’s light • Avoid alcohol and precipitant drugs • Photoprotection, antimalarials, phlebotomy

  35. Thank you

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