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
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Skin and Systemic disease
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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)
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Definition :
Erythema and scaling involving 90% or more of
the skin surface area
Erythroderma : Etiology
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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
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Erythema
Scaling
Alopecia
Nail dystrophy and shedding
Lymphadenopathy
Erythroderma: Complications
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Dehydration
Hypoalbuminemia
Loss of muscle mass
Hypothermia / Hyperthermia
Tachycardia and high- output cardiac failure
Oedema
Erythroderma: Management
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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
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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
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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
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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
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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
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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
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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
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Addison’s Disease:
Generalised, diffuse brown-black pigmentation of
skin and mucosae
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Accentuation of pigmentation on:
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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
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Contd…
Disorders of sex hormones:
Cutaneous manifestations
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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:
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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
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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
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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
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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
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Porphyrias
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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
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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
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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