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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. 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 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 Erythroderma (Exfoliative dermatitis) Definition : Erythema and scaling involving 90% or more of the skin surface area Erythroderma : Etiology Pre - existing skin disease: Psoriasis, Atopic dermatitis, Stasis dermatitis, Contact dermatitis, Seborrhoeic dermatitis, Ichthyosis, Pemphigus foliaceous, Extensive dermatophytosis, Norwegian scabies Systemic disease: Internal malignancy especially mycosis fungoides and Sezary syndrome Drugs Penicillins, Sulphonamides, Chloroquine, Sulphonylureas Idiopathic Erythroderma: Clinical features Erythema Scaling Alopecia Nail dystrophy and shedding Lymphadenopathy Erythroderma: Complications Dehydration Hypoalbuminemia Loss of muscle mass Hypothermia / Hyperthermia Tachycardia and high- output cardiac failure Oedema 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 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 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, periungual telangiectasia, erysipelas like erythema Other Skin Markers: Yellow nails, diabetic bullae, granuloma annulare, necrobiosis lipoidica, lichen planus, fat hypertrophy Diabetes Mellitus: Cutaneous manifestations Skin disorder Key features Diabetic dermopathy (shin spots) Atrophic, hyperpigmented papules/plaques on the legs very common finding Diabetic thick skin thickened skin on the hands (dorsum) - scleroderma-like changes of hands with joint stiffness - scleredema-thickening of the skin on the upper back, posterior neck, and shoulders (uncommon) Acanthosis Nigricans velvety plaques in interiginous areas (neck, axillae, groin) - common with obesity and diabetes Yellow nails and skin affects up to 50% of patients Diabetes Mellitus: Cutaneous manifestations Skin disorder Key features Acquired perforating disorders pruritic hyperkeratotic papules on the legs and trunk - histopathologically characterized by the transepidermal elimination of collagen and/or elastin Calciphylaxis deep purpuric plaques with ulceration affected patients often have diabetes and end stage renal disease with secondary hyperparathyroidism Necrobiosis lipoidica diabeticorum yellow-orange, atrophic plaques on the legs, especially the shins (Pathognomonic) Diabetic bullae spontaneous blistering of the hands and feet, heals without scarring (Pathognomonic) 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) Hyperthyroidism: Cutaneous manifestations Skin: flushed, moist, smooth, velvety to touch Hair: thin and friable; diffuse alopecia Nails: soft, friable, koilonychia, onycholysis Graves’ disease: Pretibial myxedema (waxy papules or plaques with ‘peau de orange’ look) may extend to hands, feet, thighs; Thyroid acropachy (drumstick clubbing) 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 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 acanthosis nigricans 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) 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, acanthosis nigricans Contd… 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 Renal diseases Renal failure: Persistent generalised pruritus, 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, pseudoporphyria cutanea tarda, nephrogenic fibrosing dermatopathy 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 (Henoch Schonlein, Wegener’s, Polyarteritis nodosa): Palpable purpura, vesicles, skin infarcts, ulcers Lepromatous Leprosy: Shiny papulonodules and diffuse infiltration of skin Gastrointestinal disorders Dysphagia: Rashes that may extend to esophagus Infections Congenital and acquired blistering diseases Lichen planus Behcet’s disease Stevens Johnson Syndrome Dermatomyositis Gastrointestinal disorders Bleeding: Hereditary haemorrhagic telangiectasia Blue rubber bleb nevi Ehlers Danlos syndrome Pseudoxanthoma Elasticum Kaposi’s sarcoma Gastrointestinal disorders Abdominal pain: Herpes zoster Angioedema Porphyria Anderson – Fabry disease Vasculitis: Henoch Schonlein purpura, Collagen vascular diseases Polyposis: Gardner’s syndrome, Peutz-Jeghers syndrome, ulcerative colitis, neurofibromatosis Ulcerative Colitis and Crohn’s disease Pancreatitis Gastrointestinal disorders Inflammatory Bowel Diseases: Pyoderma gangrenosum Aphthous ulcers Erythema Nodosum Malnutrition Rashes at ileostomy and colostomy sites Metastatic cutaneous Crohn’s disease Liver disease: Cutaneous manifestations Pruritus Icterus Pigmentary changes Spider angiomas Palmar erythema Dilated abdominal wall veins Purpura Loss of body hair Gynaecomastia Peripheral oedema Paraneoplastic syndromes Internal malignancies: Cutaneous Signs Acanthosis nigricans Exfoliative dermatitis Dermatomyositis Paraneoplastic pemphigus Leser-Trélat Sign: Sudden onset multiple seborrhoeic keratoses Trousseau’s sign: Migratory thrombophlebitis Sister Mary Joseph nodule Paget’s disease : underlying adnexal or breast carcinoma Acanthosis nigricans Causes: Obesity Hyperinsulinemia and diabetes Drugs: Corticosteroids, nicotinic acid, diethylstilbestrol, isoniazid Adenocarcinomas (Gastro-intestinal) Malignant acanthosis nigricans has a sudden onset and more extensive distribution: face and dorsal and palmar surfaces of the hands Sarcoidosis Non-caseating epithelioid 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) 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 Xanthomas Sign of systemic metabolic abnormality or a local cellular dysfunction or first sign of hyperlipoproteinemias Clinical Types: Eruptive, plane, tendinous, tuberous Xanthelasma palpebraerum, 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 tendinous xanthoma Porphyrias Inherited or acquired disease state where intermediate metabolites of haemoglobin synthesis (porphyrinogens) are increased Classification Erythropoietic Congenital erythropoietic Erythropoietic protoporphyria Erythropoietic coproporphyria Hepatic Acute intermittent porphyria ALA dehydratase deficiency Hereditary coproporphyria Variegate porphyria Porphyria cutanea tarda Hepatoerythrocytic porphyria Porphyria cutanea tarda 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 Porphyria cutanea tarda 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 Thank you
