Dermatological Manifestations of Systemic Diseases
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Transcript of Dermatological Manifestations of Systemic Diseases
Dermatological Dermatological Manifestations of Systemic Manifestations of Systemic
DiseasesDiseases
ByBy
Dr saad Al MohizeaDr saad Al Mohizea
Skin manifestations of Skin manifestations of Diabetes mellitusDiabetes mellitus::
1.- skin manifestations 1.- skin manifestations due to vascular due to vascular abnormalities: abnormalities:
A- A- wet gangrene of the wet gangrene of the footfoot
B- B- Diabetic BullaeDiabetic Bullae C- Diabetic C- Diabetic
dermopathy” shin dermopathy” shin spots”:spots”:
D- Erysiepilas- like D- Erysiepilas- like erythemaerythema
• 2. Diabetic 2. Diabetic neuropathy neuropathy (peripheral) (peripheral)
-Autonomic -Autonomic neuropathy neuropathy
3. 3. Cutaneous Cutaneous infections:infections:
A- A- Staphylococcus Staphylococcus aureusaureus
B- Non- clostridial B- Non- clostridial gangrene gangrene
C- Candidiasis C- Candidiasis
4. 4. Various skin disorders Various skin disorders associated with diabetes associated with diabetes mellitus:mellitus:
• A- Diabetic bullae A- Diabetic bullae
• B- Pruritus B- Pruritus
• C- Granuloma C- Granuloma annulareannulare
Skin problems associated with Skin problems associated with diabetes mellitusdiabetes mellitus
• Necrobiosis lipoidicaNecrobiosis lipoidica– Rare, insulin dependent DMRare, insulin dependent DM– One or more tender yellowish brown One or more tender yellowish brown
patches develop slowly on the lower patches develop slowly on the lower legs over several monthslegs over several months
– Round, oval or an irregular shapeRound, oval or an irregular shape– The centre of the patch becomes shiny, The centre of the patch becomes shiny,
pale, thinnedpale, thinned– Prominent blood vessels (telangiectasia)Prominent blood vessels (telangiectasia)– Often painlessOften painless
• D- Necrobiosis lipoidica D- Necrobiosis lipoidica
• Treatment: control of Treatment: control of diabetes doesn’t help in diabetes doesn’t help in disappearance of the disappearance of the lesions.lesions.– Intralesional steroids.Intralesional steroids.– Systemic aspirin: 300mg/day Systemic aspirin: 300mg/day
and dipyridamole 75 and dipyridamole 75 mg/day.mg/day.
– NicotineamideNicotineamide– Ticlopidine as antiplateletTiclopidine as antiplatelet– pentoxyfylline. pentoxyfylline. – Preilesional heparin injectionPreilesional heparin injection
- Oral cyclosporin- Oral cyclosporin
- Photochemotherapy - Photochemotherapy (PUVA)(PUVA)
Hyperlipidemia Hyperlipidemia
• XanthomataXanthomata– 4 forms : tendinous subcutaneous nodule found 4 forms : tendinous subcutaneous nodule found
in fascia, ligament and extensor tendon of in fascia, ligament and extensor tendon of hand, knee and elbowhand, knee and elbow
– Planar : yellow, soft, macule or plaque found on Planar : yellow, soft, macule or plaque found on the upper eyelidsthe upper eyelids
– Tuberous : yellow to reddish nodule at extensor Tuberous : yellow to reddish nodule at extensor surface of elbows, knees and knucklessurface of elbows, knees and knuckles
– Eruptive : sudden, multiple reddish yellow Eruptive : sudden, multiple reddish yellow papule extensor of extremities, buttockpapule extensor of extremities, buttock
PLANAR XANTHOMA
ERUPTIVE XANTHOMA
Pituitary disordersPituitary disorders::• 1. Acromegaly1. Acromegaly
– Skin is oily and wet due to Skin is oily and wet due to hyperhidrosis with wet hyperhidrosis with wet hands on hand shaking.hands on hand shaking.
– The lower lip is thickened, The lower lip is thickened, protruded with wide protruded with wide spaced teeth.spaced teeth.
– Large and furrowed Large and furrowed tongue.tongue.
– Increased skin Increased skin pigmentation.pigmentation.
– Hirsutism.Hirsutism.
2. Hypopitutirism: 2. Hypopitutirism: “Sheehan syndrome”;“Sheehan syndrome”;
• Suprarenal syndrome:Suprarenal syndrome:– Cushing syndrome: Cushing syndrome:
endogenous or endogenous or exogenousexogenous
– Deposition of fat over Deposition of fat over the clavicles and back the clavicles and back of the neck” Buffalo of the neck” Buffalo hump”hump”
– Puffy, rounded Puffy, rounded erythematosus face erythematosus face with telangiectasia with telangiectasia “Moon face”“Moon face”
– Trunkal obesity with Trunkal obesity with slender wasting limbs.slender wasting limbs.
– Striae distensae .Striae distensae .– Hirsutism, acneform Hirsutism, acneform
rash, androgenetic rash, androgenetic alopecia.alopecia.
– Addisonian-like Addisonian-like pigmentation pigmentation
– Easy bruising of the Easy bruising of the skin on simple traumaskin on simple trauma..
Striae distensaeStriae distensae • Aetiology:Aetiology:
• Idiopathic Idiopathic
• pregnancy: Striae pregnancy: Striae gravidarumgravidarum
• Endocrine: cushingEndocrine: cushing
• Iatrogenic: Iatrogenic: systemic or potent systemic or potent topical steroids, topical steroids, anabolic drugs or anabolic drugs or androgens.androgens.
• Hypocorticism Hypocorticism ”Addison,s disease”: ”Addison,s disease”: Generalized Generalized hyperpigmentation hyperpigmentation that is more prominent that is more prominent in light exposed areas, in light exposed areas, scars, genitalia, scars, genitalia, palmar and finger palmar and finger creases, and under the creases, and under the nails. The nails. The pigmentation pigmentation characteristically characteristically affects the mucous affects the mucous membranes.membranes.
• Loss of pubic and Loss of pubic and axillary hair in axillary hair in females.females.
• Improvement of acne.Improvement of acne.
• IV- Thyroid diseases:IV- Thyroid diseases:• Hyperthyroidism:Hyperthyroidism:• red,soft, moist and hot skin.red,soft, moist and hot skin.• addisonian addisonian
hyperpigmentation i.e. not hyperpigmentation i.e. not affect the mucous affect the mucous membranes.membranes.
• Diffuse thinning of scalp hairDiffuse thinning of scalp hair• Rapid nail growth and Rapid nail growth and
onycholysis.onycholysis.• Generalised pruritus and Generalised pruritus and
urticaria.urticaria.• Palmar erythema and facial Palmar erythema and facial
flushing.flushing.• Hyperhidrosis or increased Hyperhidrosis or increased
sweating.sweating.
• Pretibial Pretibial myxoedema: myxoedema: appears as small red appears as small red or skin colored or skin colored coalescingcoalescing
NodulesNodules
• Thyroid acropatchy Thyroid acropatchy
Endocrinologic DiseasesEndocrinologic Diseases
• HypothyroidismHypothyroidism
MacroglossiaMacroglossia
• Hypothyroidism:Hypothyroidism:
• Buffy face with coarse Buffy face with coarse features.features.
• The skin is pale, The skin is pale, thickened, cold, finely thickened, cold, finely scaling and wrinkled.scaling and wrinkled.
• Coarse sparse hair of Coarse sparse hair of the scalp with loss of the scalp with loss of the lateral third of the the lateral third of the eyebrows.eyebrows.
• Brittle and striated hair.Brittle and striated hair.
• Poor wound healing.Poor wound healing.
• Decreased or absent Decreased or absent sweating.sweating.
Gastrointestinal tract and Gastrointestinal tract and the skinthe skin
• Crohn,s disease Crohn,s disease ”regional ileitis””regional ileitis”
• Perianal abscess and Perianal abscess and fisulae.fisulae.
• Erythema nodosumErythema nodosum• Erythema multiformeErythema multiforme• Aphthous-like stomatitis Aphthous-like stomatitis
and glossitis.and glossitis.• Cutaneous vasculitis.Cutaneous vasculitis.• Epidermolysis bullousa Epidermolysis bullousa
acquisita.acquisita.• Metastatic Crohn,s Metastatic Crohn,s
disease as numerous disease as numerous eroded cutaneous eroded cutaneous granulomas at sites granulomas at sites distant from the affected distant from the affected intestine.intestine.
• Pyoderma gangrenosum.Pyoderma gangrenosum.
Pyoderma gangrenosumPyoderma gangrenosum • Aetiology:Aetiology:• Idiopathic in 50% of the cases.Idiopathic in 50% of the cases.• Inflammatory bowel disease- Inflammatory bowel disease-
Crohn,s disease and ulcerative Crohn,s disease and ulcerative colitis.colitis.
• Connective tissue diseases- Connective tissue diseases- Rheumatoid arthritis, SLE or Rheumatoid arthritis, SLE or Behc,et disease.Behc,et disease.
• Blood disease: Leukemia, multiple Blood disease: Leukemia, multiple myeloma, polycythemia or myeloma, polycythemia or monoclonal gammopathy.monoclonal gammopathy.
• Treatment:Treatment:– Dealing with underlying cause.Dealing with underlying cause.– Systemic steroids in high dose Systemic steroids in high dose
like 60-80 mg oral prednisolone like 60-80 mg oral prednisolone daily and then reduce daily and then reduce gradually.gradually.
• Azathioprime, Dapsone, or Azathioprime, Dapsone, or cyclosporinecyclosporine
• Ulcerative colitisUlcerative colitis: : identical to identical to Crohn,s disease but pyoderma Crohn,s disease but pyoderma gangrenosum and erythema gangrenosum and erythema nodosum are more common in nodosum are more common in ulcerative colitis.ulcerative colitis.
• MalabsorptionMalabsorption: specific or : specific or non-specific features.non-specific features.
• Non- specific featuresNon- specific features: : Pallor, dry skin, oedema, Pallor, dry skin, oedema, acquired icthyosis, acquired icthyosis, Pigmentary disorders, Pigmentary disorders, glossitis, mouth ulcers.glossitis, mouth ulcers.
• Specific featuresSpecific features due to due to certain factor deficiency:certain factor deficiency:
• e.g. – Follicular e.g. – Follicular hyperkeratosis --------------- hyperkeratosis --------------- Vit. A deficiency.Vit. A deficiency.
• Echymosis and scurvy Echymosis and scurvy ------------------ Vit. C ------------------ Vit. C deficiency.deficiency.
• Acrodermatitis Acrodermatitis enteropathica ---------- Zinc enteropathica ---------- Zinc deficiency.deficiency.
• Liver diseases:Liver diseases:
• Pruritus: generalized Pruritus: generalized itching especially in the itching especially in the presence of biliary presence of biliary obstruction or jaundice.obstruction or jaundice.
• Urticaria especially in Urticaria especially in infectious hepatitis.infectious hepatitis.
• Jaundice.Jaundice.• Spider naevi: small Spider naevi: small
telangeictatic blood telangeictatic blood vessels especially on the vessels especially on the face and upper chest.face and upper chest.
• Palmar erythema.Palmar erythema.• Diffuse Diffuse
hyperpigmentationhyperpigmentation..
Thinning of the hair and Thinning of the hair and sometime loss of sexual sometime loss of sexual hair in the axillae and hair in the axillae and pubic areas.pubic areas.
Gynaecomastia.Gynaecomastia.
Acneform-lesions.Acneform-lesions.
Porhyria cutanea tarda.Porhyria cutanea tarda.
Xanthoma: collection of Xanthoma: collection of lipid in the dermal tissue lipid in the dermal tissue especially in primary especially in primary biliary cirrhosis.biliary cirrhosis.
Behcet diseaseBehcet disease
• Recurrent aphthous oral Recurrent aphthous oral ulcerations more than ulcerations more than three times per a year is a three times per a year is a must plus two of the must plus two of the following:following:
• Genital ulceration or other Genital ulceration or other skin manifestations skin manifestations Erythema-nodosum like Erythema-nodosum like lesions, pseudofolliculitis, lesions, pseudofolliculitis, papulopustular lesions or papulopustular lesions or acneform rash.acneform rash.
• Eye lesions: anterior or Eye lesions: anterior or posterior uveitis posterior uveitis
• Positive pathergy test Positive pathergy test
Generalised pruritusGeneralised pruritus
• Generalised pruritus in the absence Generalised pruritus in the absence of a rash requires investigation and of a rash requires investigation and exclusion of an underlying systemic exclusion of an underlying systemic disorder disorder
• it is important to distinguish these it is important to distinguish these from an underlyingfrom an underlying
• primary skin disease such as scabies primary skin disease such as scabies or eczemaor eczema
Erythema nodusomErythema nodusom
sarcoidosissarcoidosis
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DMDM
Cutaneous markers of Cutaneous markers of internal malignancyinternal malignancy
• I.Genetically I.Genetically determined syndromes:determined syndromes:
Gardners syndrome, Gardners syndrome, Howel-Evans syndrome, Howel-Evans syndrome, Torre-Muir syndrome.Torre-Muir syndrome.
• II. II. II. Skin signs of II. Skin signs of exposure to carcinogen:exposure to carcinogen:
e.g. – Nicotine staining, X-e.g. – Nicotine staining, X-ray damage ray damage
Paraneoplastic Paraneoplastic dermatosesdermatoses
• 1. Acanthosis 1. Acanthosis nigricans nigricans
• 2. Dermatomyositis 2. Dermatomyositis
• 3. Erythema 3. Erythema gyratum repens gyratum repens
• 4. Erythema 4. Erythema nodosum and nodosum and erythema erythema multiforme multiforme
• 5. Acquired icthyosis5. Acquired icthyosis
• 6. Pruritus 6. Pruritus
• 7. Bullous eruptions 7. Bullous eruptions
• 8. Pyoderma 8. Pyoderma gangrenosumgangrenosum
• 9. Erythroderma 9. Erythroderma
• 10. Flushing of the face10. Flushing of the face • 11. Urticaria11. Urticaria
12. Acquired hypertrichosis 12. Acquired hypertrichosis lanuginosalanuginosa
• 13. Disseminated herpes zoster 13. Disseminated herpes zoster
• 14. Migratoratory superficial 14. Migratoratory superficial thrombophlebitis thrombophlebitis
• 15. Hypertrophic osteoarthropathy 15. Hypertrophic osteoarthropathy and Finger clubbing and Finger clubbing
• 16. Tripe palms 16. Tripe palms
• 17. The sign of Leser-Telat 17. The sign of Leser-Telat
• 18. Necrolytic migratory erythema 18. Necrolytic migratory erythema
• IV. Direct metastases to IV. Direct metastases to the skin e.g. Carcinoma of the skin e.g. Carcinoma of the breast or lymphoma.the breast or lymphoma.
Sign of Leser-TrelatSign of Leser-Trelat
• Sudden appearance of multiple Sudden appearance of multiple seborrheic keratosesseborrheic keratoses
• Association with internal malignancy Association with internal malignancy is unproven is unproven
•18. 18. Necrolytic Necrolytic migratory migratory erythemaerythema
Nephrogenic fibrosing Nephrogenic fibrosing dermopathydermopathy• fibrotic disease of the skin and internal fibrotic disease of the skin and internal
organs that develops in patients with organs that develops in patients with renal insufficiency after exposure to renal insufficiency after exposure to gadolinium-based contrast agents. It is gadolinium-based contrast agents. It is similar to, yet distinct from, scleroderma. similar to, yet distinct from, scleroderma.
• pain and pruritus.pain and pruritus.
• Most patients are on hemodialysis or Most patients are on hemodialysis or peritoneal dialysis or have had a renal peritoneal dialysis or have had a renal transplantation. transplantation.
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Cutaneous small vessel Cutaneous small vessel vasculitisvasculitis• mainly cutaneous post-capillary mainly cutaneous post-capillary
venules, venules,
• 50% of cases ‘idiopathic’,50% of cases ‘idiopathic’,
• 15% due to infection, 15% due to infection,
• 15% related to inflammatory diseases 15% related to inflammatory diseases (such as collagen vascular disorders),(such as collagen vascular disorders),
• 15% due to medications 15% due to medications
• 5% are associated with malignancy5% are associated with malignancy
Leukocytoclastic vasculitis Leukocytoclastic vasculitis
• may be localized to the skin or may may be localized to the skin or may manifest in other organs. The manifest in other organs. The internal organs affected most internal organs affected most commonly include the joints, the commonly include the joints, the gastrointestinal tract, and the gastrointestinal tract, and the kidneys. The prognosis is good in the kidneys. The prognosis is good in the absence of internal involvement. absence of internal involvement.
PhysicalPhysical• The most common manifestation of cutaneous The most common manifestation of cutaneous
vasculitis is palpable purpura, but other vasculitis is palpable purpura, but other manifestations may develop.manifestations may develop.
• They may coalesce to form plaques; in some They may coalesce to form plaques; in some instances, they may ulcerate.instances, they may ulcerate.
• Legs is most common locationLegs is most common location
• Urticarial lesions Urticarial lesions
• Livedo reticularisLivedo reticularis
• Nodular lesionsNodular lesions
• UlcerationUlceration
RxRx• treatment of cause. treatment of cause.
• colchicine or dapsonecolchicine or dapsone
• antihistaminesantihistamines
• NSAIDSNSAIDS
• severe visceral involvement may require high doses of corticosteroids severe visceral involvement may require high doses of corticosteroids with or without an immunosuppressive agentwith or without an immunosuppressive agent
• severe or debilitating disease might also be treated with biologic agents severe or debilitating disease might also be treated with biologic agents such as rituximab or intravenous immunoglobulinsuch as rituximab or intravenous immunoglobulin
vasculitisvasculitis