الجلدية والأمراض الداخلية .. Skin manifestations of internal diseases

HEART AND SKIN

Familial hyperlipidemia:Increased risk of coronary heart disease. Xanthomatosis may be present in thesedisorders

Cholesterol emboli:In patients with advanced atherosclerosis of the abdominal aorta, cholesterol crystalsmay microembolize to the lower extremities. Pulse may remain normal, patients maydevelop livedo reticularis (reticulated erythematous/vascular pattern) on lower legs,abdomen, with focal cyanosis, crusting and gangrenous changes

Subacute bacterial endocarditis (SBE): Vasculitic lesions (Oslers nodes and Janeway'slesions) appearing as purpuric palpable lesions on finger tips, palms and soles. Alsosplinter hemorrhages (not specific). Petechiae and larger purpuric lesions may bemore widespread. Pathogenesis may be immunologic or septic vasculitis

SKIN SIGNS OF INTERNAL MALIGNANCY

Syndromes -some genetically determined with a cutaneous component and aninherent predisposition to internal malignancy

Gardner's syndrome
Peutz - Jegher's syndrome
Muir-Torre syndrome
Cowden's syndrome
Neurofibromatosis
Mucosal neuroma syndrome

Cutaneous markers of exposure to a carcinogen capable of inducinginternal malignancy

Arsenic
Nicotine staining
X-ray damage -radiodermatitis

Proliferative, inflammatory, deposition and other skin changes associatedwith internal malignancy

Pruritus
Erythema gyratum repens
Sweet's syndrome
Hypertrichosis lanuginosa
Necrolytic migratory erythema
Clubbing
Eruptive seborrheic keratoses
Dermatomyositis -adult
Acanthosis nigricans
Acquired ichthyosis
Flushing
Thrombophlebitis
Telangiectasia, palmar erythema
Deposition -jaundice,melanosis,hemochromatosis,xanthomas,syste micamyloidosis
Vascular hypersensitivity reaction (e.g. vasculitis, erythema multiforme
Palmar and plantar keratoderma


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 serum

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
Rx: prednisone

Trousseau’s sign

superficial migratory thrombophlebitis and neoplasia
75% of CA in pancreas, stomach, lung, prostate, hematopoietic
hypercoagulable state; thrombophlebitis resistant to therapy--sustained low grade DIC

Pancreatic panniculitis

Fat necrosis, fever, eosinophilia, joint pain
Pancreatic CA; also acute or chronic pancreatitis
Circulating lipases and amylase
Tender red nodules stimulating erythema nodosum

heritable cancer syndromes

Peutz-Jehger
Torre
Cowden’s
etc

Peutz-Jehger syndrome

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

Torre syndrome

autosomal dominant; "cancer-family" syndrome
multiple low-grade malignancies of the GI and GU tracts; occasionally breast, lymphoma; multiple GI polyps in 25%
sebaceous neoplasms: adenomas, carcinomas, sebaceous BCCs

Skin Manifestations of Systemic Disease Drug eruptions

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



severe erythema multiforme affecting mucous membranes as well as skin is called "Stevens-Johnson syndrome



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

Skin signs of internal disease other than malignancies

Pyoderma gangrenosum


characteristic 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 disease associations: Crohn’s, chronic active hepatitis, rheumatoid arthritis, HIV infection; acute and chronic granulocytic leukemia -bullous PG
can be 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

Porphyria cutanea tarda

most common porphyria; sporadic>80%; autosomal dominant in remainder
photosensitivity; skin fragility with blistering, scarring and milia
facial hypertrichosis; scleroderma-like change

Porphyria cutanea tarda -cont

defect: uroporphyrin decarboxylase in liver and erythroctyes; increased iron stores
ETOH, estrogen, iron, hexachlorobenzene toxicity
Rx: phlebotomy, antimalarials

Xanthomas

may be 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 are xanthomas of eyelids that may or may not be associated with hyperlipidemia

eruptive xanthomas, tendon xanthomas, and tuberous xanthomas are signs of significant hyperlipidemia; these patients require careful evaluation and prompt treatment

tendon xanthomas

extensor tendons of fingers, patella, elbows, Achilles tendon (one of the most common sites); diffuse infiltration of tendon by lipid
hypercholesterolemia; Types II and III
normal lipids: cerebrotendinous xanthomatosis; plant sterols


tuberous xanthomas

lipid deposits in the dermis and subcutis; papuler, nodular or plaques; extensor surfaces of large joints, hands, buttocks, heels, flexures
familial or acquired hypertriglyceridemias; biliary cirrhosis



other xanthomas

Eruptive--small reddish-yellow papules; buttocks, posterior thighs, body folds


usually abrupt increase in serum triglyceride levels
Plane xanthomas--flat yellow plaques on palms, face, lateral neck, upper trunk; xanthelasma of eyelids; seen in biliary cirrhosis
familial type III and type IV

Acanthosis nigricans -AN

velvety thickening and darkening (hyperpigmentation) of the skin, especially on the nape of the neck, axillae and other body folds




underlying causes may be hereditary or acquired, and include
obesity; drugs; "malignant" acanthosis nigricans; hereditary, benign AN
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



Telogen effluvium

a distinctive form of hair loss that is a response to an underlying systemic condition
normally, about 50-100 telogen hairs are shed from the scalp each day; in telogen effluvium, this number is greatly increased
the telogen hairs start falling out about 3 months after a "precipitating event--" major surgery, severe illness, certain drugs, and childbirth being the most common. Chronic disease or drug ingestion can cause a sustained telogen effluvium--e.g., hypothyroidism, retinoid use


Erythema chronicum migrans -erythema migrans

often the first manifestation of Lyme disease = Lyme borreliosis
spirochete Borrelia burgdorferi is transmitted by the bite of the deer tick Ixodes scapularis
systemic borreliosis is a potentially serious disease, causing both acute and chronic symptoms such as fever, malaise, arthralgia, carditis, arthritis, meningitis, etc

Erythema chronicum migrans -erythema migrans

typical lesion is a macule or papule that expands over several days, with central clearing, to form an annular, erythematous patch or plaque; may reach 15 or more CM in size
soldiers and Marines hiking through fields in endemic areas are prone to this disease

Chronic, cutaneous lupus erythematosus -discoid lupus erythematosus = DLE


DLE may be one of several cutaneous findings seen in systemic lupus erythematosus
note: DLE often occurs as an isolated finding, not associated with SLE
sharply marginated, scaly, atrophic, red plaques; round, oval or polycyclic
more common in young adult women
exposed areas of body: face, scalp, ears, hands, forearms

Leukocytoclastic vasculitis -LCV

clinically causes "palpable purpura," small, raised areas of cutaneous hemorrhage and inflammation at the site of venular destruction
may be associated with fever, joint pain, and internal organ damage -kidneys, GI tract, brain

lesions are usually numerous and tend to affect the legs and ankles most severely

multiple underlying "causes" can precipitate LCV

drugs, such as sulfonamides, penicillins, others
infections, such as Group A streptococcal, viral hepatitis (e.g. Hep. C), others
immunologic diseases such as systemic lupus erythematosus, rheumatoid arthritis, cryoglobulinemia
neoplasms such as lymphomas
idiopathic--no cause found in @50% of cases

Behcet’s disease

triad of oral and genital ulcerations and inflammatory eye disease
males 2:1; HLA-B5 increased in some populations
onset with aphthous ulcers and ulcers of scrotum or labia
fever, malaise, arthralgia, iritis, uveitis
pathergy; erythema nodosum; thrombophlebitis; CNS disease--poor prognosis

Reiter’s syndrome

post-venereal and post-enteric; HLA-B27
urethritis, conjunctivitis, iritis, arthritis
skins lesions: keratoderma blenorrhagicum (keratotic conical lesions on lateral and palmoplantar aspects of hands and feet)
circinate balanitis--annular erythematous lesions on glans penis
psoriasiform lesions on scrotum, buttocks, trunk, extremitis, scalp
marked nail dystrophy, painful erythema of fingers and toes

أحبـائي الأعـزاء

قمـت بنقـل المـوضـوع

من عـدة صفحـات عبر الانترنت

أتمنى أن ينـال إعجـابـكم يحـوز على رضـاكم

كمـا آمـل أن ينتفـع منه الكثيرين

شـاكرة لكـم تـواصـلكم الذي سيسعدني دائمــاً

ويدفعني لتقديم المزيـد لإرضـاء أذواقكـم الـراقيـة





محبتي لجميعكـم

angelbird


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