Skin Manifestations Of Systemic Diseases

SKIN MANIFESTATIONS OF SYSTEMIC DISEASES BY Professor M.YOUSRY ABDEL_MAWLA

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

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
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..)

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)

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

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