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Skin Manifestations Of Systemic Diseases
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Skin Manifestations Of Systemic Diseases



skin manifestations of systemic diseases

skin manifestations of systemic diseases



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Skin Manifestations Of Systemic Diseases Skin Manifestations Of Systemic Diseases Presentation Transcript

  • Skin Manifestations of Systemic Disease
    • Skin signs of internal malignancy
    • Skin signs of internal disease other than malignancy
    • Drug eruptions
  • Skin signs of internal malignancy
    • cutaneous metastases
    • paraneoplastic syndromes
    • heritable "cancer syndromes"
  • Sister Mary Joseph nodule
    • Umbilical metastasis; poor prognosis
    • precedes or follows diagnosis of CA
    • CA sites (decreasing order of frequency): colon, ovary, pancreas, endometrium, breast, small bowel
  • Paraneoplastic Syndromes
    • The skin often presents a clue that an internal malignancy is present. The combination of a malignancy and associated signs and symptoms that are seemingly unrelated to the actual tumor is called a "paraneoplastic" syndrome
  • Erythema gyratum repens
    • "Wood-grain" pattern
    • Wavy, erythematous, urticarial bands with scale
    • Slowly migrate
    • Breast, stomach, bladder, prostate, cervix; occasionally no CA
  • Glucagonoma syndrome -- necrolytic migratory erythema
    • Alpha cell tumor of the pancreas; occasionally no neoplasm found
    • abdomen, thighs and buttocks
    • Patchy erythema with flaccid vesicles and bullae
    • Glossitis, angular cheilitis, normocytic anemia, low amino acid levels in seum
  • Sweet’s syndrome (acute febrile neutrophilic dermatosis )
    • Painful red plaques and papules - face, neck, upper chest, arms, legs
    • Usually females (4:1); fever, leukocytosis prominent
    • Associations, benign: URIs, strep, RA, Crohns, sarcoidosis, Behcet’s, pregnancy
    • Association, malignant: AML, myelodysplasis, lymphoma--may follow by months to years
  • Trousseau’s sign
    • Superficial migratory thrombophlebitis and neoplasia
    • 75% of CA in pancreas, stomach, lung, prostate, hematopoietic
    • Hypercoagulable state; thrombophlebitis resistant to therapy .
  • heritable "cancer syndromes
    • Peutz-Jehger
    • Multiple hamartomatous polyps in small bowel (most common), stomach and colon; low risk of bowel malignancy
    • multiple lentigines (freckle-like) of lips, nose, oral mucosa, fingertips and nail beds
    • non-intestinal malignancies increased: lung, ovary, endometrium pancreas, myeloma
  • Skin signs of internal disease
    • Pyoderma gangrenosum
    • Cullen’s sign
    • Xanthomas
    • Acanthosis nigricans (AN)
    • Erythema nodosum
  • Pyoderma gangrenosum
    • Rapidly expanding ulcer with bluish undermined border; often lower extremities; begin as sterile pustules
    • 50% no disease association
    • 1% to 10% of patients with active ulcerative colitis; often (but not always) parallels disease
    • Other associations: Crohn’s, chronic active hepatitis, rheumatoid arthritis, HIV infection; acute and chronic granulocytic leukemia (bullous PG)
    • Associated with underlying malignancy (leukemia, etc.)
  • Cullen’s sign
    • Periumbilical purpura associated with acute pancreatitis
    • Hematomas dissect along fascial plans from the retroperitoneal site of bleeding to the periumbilical area
    • Turner’s sign- -purpura of the left flank; same cause
  • Xanthomas
    • A sign of systemic metabolic abnormality or a local cellular dysfunction
    • Xanthomas may be the first sign of one the hyperlipoproteinemias, rare but serious metabolic diseases
    • Xanthelasma : xanthomas of eyelids that may or may not be associated with hyperlipidemia
  • Acanthosis nigricans (AN)
    • Velvety thickening and darkening (hyperpigmentation) of the skin, especially on the nape of the neck, axillae and other body folds  
    • Causes
    • hereditary
    • acquired, : obesity; drugs; "malignant"acanthosis nigricans; Diabetes Mellitus
    • hyperinsulinemia is a common denominator
  • Erythema nodosum
    • Deep erythematous painful nodules, symmetrically on the lower legs; female predominance; a hypersensitivity panniculitis
    • Fever, chills, malaise, leukocytosis
    • Disease associations: streptococcal infections, drugs (OCPs, sulfonamides, iodides), pregnancy, TB, deep mycoses, acute sarcoidosis, inflammatory bowel disease
  • Skin Manifestations of other Systemic Diseases
    • Gastrointestinal Disease
    • Diabetes mellitus
    • Liver disease
    • Kidney Diseases
    • Other disorders
    • Sarcoidosis
    • Leucocytoclastic vasculitis in SLE
  • Gastrointestinal Disease
    • Inflammatory Bowel Disease
    • Pyoderma gangrenosum
    • Erythema nodosum
    • Aphthous stomatitis .
    • Mucosal nodularity (cobblestoning).
    • Pyostomatitis vegetans (erythematous thickened mucosa)
    • Gastrointestinal disorders Cutaneous disorders
    • Inflammatory bowel disease
    • Ulcerative colitis Neutrophilic tissue reactions
    • Crohn’s disease Pyoderma gangrenosum
    • Bowel – bypass syndrome Sweet’s syndrome
    • Pustular vasculitis
    • Erythema nodosum
    • Aphthous stomatitis
    • Malabsorption
    • Gluten-sensitive enteropathy
    • Alcoholic liver disease
    • Acrodermatitis enteropathica
    • Dermatogenic enteropathy
    • Pancreatic panniculitis
    • Porphyria cutanea tarda
    • Dermatitis herpetiformis
    Nutritional and Metabolic disorders
  • Infections
    • Hepatitis B and C
    • Helicobacter pylori
    • Porphyria cutanea tarda
    • Erosive or oral lichen planus
    • Sweet's syndrome
  • Malignancies
    • Glucagonoma
    • Carcinoid
    • Esophageal carcinoma  
    • Nonspecific cutaneous signs of gastrointestinal malignancy
    • Necrolytic migratory erythema
    • Flushing
    • Bazex's syndrome
    • Palmar-plantar hyperkeratosis
    • Koilyonychia
    • Glossitis
    • Acanthosis nigricans
    • Erythema nodosum
    • Metastatic skin lesions (eg adenocarcinoma of colon
    • Sister Mary Joseph's nodules)
  • Malignancies
    • Gastrointestinal polyposis syndromes and gastrointestinal cancer
    • Peutz-Jeghers syndrome
  • Gastrointestinal hemorrhage
    • Vascular disorders
    • Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease)
    • Kaposi's sarcoma
    • Necrotizing angiitis
    • Pseudoxanthoma elasticum
    • Ehlers-Danlos syndrome
    • Degos, disease
    • Henoch-Schönlein purpura
  • Dermatologic Manifestations of liver disease
    • Vascular
    • a. Spider telangiectasia,
    • b. Palmer erythema,
    • c. Corkscrew scleral vessels,
    • d. Caput medusae,
    • e. Plethoric facies,
    • f. Flushing,
    • g. Unilateral nevoid telangiectasia.
    • Jaundice,
    • Pruritus,
    • Urticaria,
    • Nail changes –
    • Koilonychia, Clubbing.
    • Hyperpigmentation ,
    • Skin cancer,
    • Lichenoid dermatitis,
  • Skin manifestations of Renal diseases
    • Uremic pruritus :generalized or localized
    • Excoriations, lesions of lichen simplex chronicus, and prurigo nodularis.
    • Mediated by various chemical mediators neurotransmitters: (pentapeptide enkephalins, proteases,
    • serotonin, and histamine..)
  • Skin manifestations of Renal diseases
    • 2-Xerosis : the dry or roughened skin texture
    • A direct relation between xerosis and stratum corneum water content.
    • A thicker though fractured stratum corneum leads to increase transepidermal water loss.
    • 3-Cutaneous pigmentation:pallor , brown-to-slate-gray discoloration, yellowish (sallow) hue, and brownish hyperpigmentation in sun-exposed areas.
    • Due to increased levels of melanocyte stimulating hormone (MSH)
  • Skin manifestations of Renal diseases
    • 4-Calciphylaxis: a life-threatening condition of progressive cutaneous necrosis due to small- and medium-sized vessel calcification.
    • A complication of secondary hyperparathyroidism in chronic renal failure.
    • Firm, bilaterally symmetric non-ulcerating painful
    • preinfarctive ischemic plaques that appear as mottling or violaceous discoloration on the extremities developing into necrotic areas. ---_Flaccid or hemorrhagic bullae may form over ischemic tissue
  • Diabetes mellitus
    • Vascular microangiopathies and rubeosis
    • Recurrent skin infections :, either due to fungi (e.g. genital candidiasis ) or bacteria (e.g. folliculitis).
    • Blisters on the feet
    • Wet gangrenes
    • Peripheral neuropathy and trophic skin changes
    • Brown macules sometimes develop on the shin
    • Plaques with dark red or purple edges, atrophic centres, and surface telangectasia (Necrobiosis lipoidica diabeticorum ).
  • Drug eruptions
    • Urticaria
    • Morbilliform
    • Fixed drug eruption
    • Erythema multiforme
    • Stevens-Johnson Syndrome
    • Toxic epidermal necrolysis
  • Urticaria (hives)
    • Drugs (e.g., penicillins) are a common cause of urticaria, but urticaria can be precipitated by other internal and external factors
    • Primary lesion is a wheal, a flesh-colored to pink, well circumscribed plaque caused by dermal edema; itchy!
    • Individual lesions last only a few hours, never more than 24 hours
    • When caused by drugs, may be IgE mediated, triggering mast cell granule release; or drug may directly cause mast cell granule release
  • Morbilliform eruption (exanthematous drug eruption, maculopapular drug eruption) :  
    • "morbilliform" refers to a resemblance to the rash of measles (morbilli is Latin for measles); measles is a rare disease now, but morbilliform eruptions are common
    • a morbilliform eruption is symmetrically distributed on the trunk and proximal extremities, and consists of bright pink macules and slightly raised papules ("maculopapular")
  • Fixed drug eruption
    • Fixed" in that it occurs at same sites with each episode
    • OTC drugs containing phenolphthalein, pseudoephedrine, etc. common culprits
    • tetracyclines, barbiturates, phenothiazines, sulfonamides
    • oval, itchy or burning dusky red plaque
  • Erythema multiforme
    • A form of cutaneous reaction to an underlying condition. In 50% of cases, a cause can’t be identified
    • Common causes :
    • Drugs (sulfonamides, phenytoin, barbiturates, penicillin, etc.); infections (esp. herpes simplex and Mycoplasma); inflammatory bowel disease
    • Eruption usually lasts for a week or two, then spontaneously remits
    • the "target" lesion is approximately 1cm dull-red macule or papule with a central area of blistering or hemorrhage
  • Toxic epidermal necrolysis (TEN)
    • it is unclear whether TEN is a severe form of erythema multiforme or a distinct disease
    • 80% of cases have a strong association with a specific drug (list is similar to that for erythema multiforme)
    • TEN is a medical emergency, on the order of a total body burn