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Cutaneous Manifestations of Systemic Diseases
Dr. Halder
4/27/04 8am
He says that he wont talk much about the actual diseases but will focus on their
cutaneous manifestation. He basically taught the same thing as he did last year so I
added to the 2006 notes. If it is in Red, he didnt talk about it this year in detail, but I left
it because he mentioned some of them with other manifestations.
DIABETES
Necroboisis lipoidica (diabeticorum) NL
A better term to use now is necrobiosis lipoidica, leaving the diabetic part off because
diabetes is not always associated with this condition. In fact, in 30% of patients, its not
associated with diabetes. 50% have no underlying diabetes.
Can occur before or after the onset of diabetes. In about 10-14% of patients, it will occur
before the onset of diabetes.
Usually on the lower extremities anterior tibial surfaces
Reddish, waxy, somewhat firm, shiny areas, may have some degree of atrophy and its
normally bilateral. Somewhat firm, and some may have ulceration
Due to microangiopathy. This is the same microangiopathy that is responsible for
manifestations of diabetes in other organs (like eye, skin, kidneys, and many other
organs)
Eruptive Xanthomas
Depositions of cholesterol and triglycerides in the skin. They occur as papules.
Papules are skin colored. If the patient is lighter skinned, they can be salmon or pink
colored.
Occur very suddenly. You will see the onset of all these lesions, and a patient will tell
you that these lesions have occurred over a period of weeks or days
The importance of eruptive xanthoma is that it is usually associated with new onset
diabetes. These patients are relatively youngthey tend to be males, often in their 20s
o Blood sugar will be extremely high. Anywhere from 300-500.
This is definitely a marker for new onset diabetes
Once they get their diabetes under control, these lesions will resolve
THYROID DISEASE
Periorbital Myxedema (Myxedema)
Associated with hypothyroidism
You get swelling of both eyelids, bilaterally. Both upper and lower.
With hypothyroidism, there are other skin and hair manifestations (dry skin, itchy skin,
dry hair, and brittle hair)
Pretibial Myxedema
Pretibial area
Due to Hyperthyroidism

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They are shiny, and they are depositions of mucopolysaccharide- firm, large confluent
plaques on the lower legs.
Due to production of thyroxine/ LATS (long acting thyroid stimulator) fibroblasts
in the skin in this anatomical area. We dont know why it only stimulates them
here.
Treatment of the underlying hyperthyroidism may or may not cause these lesions to
resolve
Other manifestations of hyperparathyroidism in the skin
o hair loss (can be with hyper and hypothyroid)
o The skin itself will be very moist and patients will have increased temperature of
the skin. So the skin in hyperthyroid is warm and moist, and its dry in
hypothyroidism
AUTOIMMUNE DISEASE

Vitiligo
Presumed to be an autoimmune disorder of the skin. It is due to anti-melanocyte
antibodies. SO the patient is producing antibodies that are specifically attacking
melanocytes and destroying them. Thats why in majority of cases, its an autoimmune
condition.
Usually seen in middle aged women
As with other endocrine and autoimmune diseases, treating the underlying disease does
not improve the vitiligo
Another example of vitiligo, Acral (on the hands) vs. segmental (usually unilateral and
seen in children, with a dermatomal distribution). The children usually dont have the
same association with autoimmune disorders as seen in adults.
Because it is considered and autoimmune disorder, it is also associated with other
autoimmune diseases, and approximately 15% of patients with vitiligo will go on to have
these other autoimmune conditions. The common ones include:
o Diabetes (can be juvenile onset or it can be adult onset)
o Addison disease
o Thyroid diseases (particularly hyperthyroidism)
o Pernicious anemia
Acanthosis Nigricans
Described as velvety hyperpigmented areas in flexural areas of the body, particularly in
the axilla and around the neck
There are several types of acanthosis nigricans
o Malignant The skin does not have malignancies. This type of acanthosis
nigricans is associated with internal malignancies, particularly malignancies of
the GI tract
o Metabolic Associated with insulin resistant diabetes and thyroid disease
o Benign associated particularly in obese adolescent females. Not associated
with any internal disease. We see this a lot with our patient population. For some
reason, this type occurs more often in skin of color.
o Hereditary- occurs in African-Americans, Hispanics, and some Asians
Picture: This patient has acanthosis nigricans on the oral mucosa and on the tongue.
This is very unusual.

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Pyoderma Gangrenosum
The term pyoderma does not necessarily mean that this is a bacterial infection. Thats
really a misnomer
This is an inflammatory condition of the skin in which you have ulcerations, particularly
on the lower extremities.
Characteristically with these ulcerations, you will have granulomatous formation that you
can see in the center, but in the periphery you see that theres a dark purple border
around the whole lesion. This is typical of pyoderma gangrenosum
Associated with other autoimmune diseases
o Rheumatoid arthritis
o Inflammatory Bowel Disease
The activity of the pyoderma gangrenosum can be correlated with the underlying
disease. So if the Rheumatoid arthritis is flaring up, the pyoderma gangrenosum gets
worse. Same with the IBD when the underlying disease is treated, this tends to do
better
This is NOT an infection of the skin!
NEUROCUTANEOUS DISEASES
(Affect the nervous system),
Tuberous Sclerosis
Adenoma Sebaceum
o Small, firm, Inflammatory papules, particularly on the face (found around the
nose, around the mouth, and on the chin). Looks very much like acne. Patients
develop these at a much earlier age then they develop acne. SO you will see
these in childhood versus adolescence.
o It may look like acne, but theyre fibrotic papulesits fibrous tissue. They are
not pustular
Other skin findings of TS:
o Ash leaf macule occurs at birth. These are hypopigmented lesions (almost
white), they have the shape of an ash leaf. Kind of an oval appearance. Is quite
common and very characteristic in diagnosing tuberous sclerosis.
o Subungal fibromas fibrotic tumors that occur under the nails
o Caf Au Lait spots These are dark brown to tan macules that can occur
anywhere on the skin
Tuberous sclerosis can also be assoc with seizure disorders, Subungal Fibromas, Renal
Tumors, and mental retardation
Neurofibromatosis (von Recklinghousens Disease)
The common skin findings are these tumors that we call neurofibromas. They can be
very large, some are smaller, and they can be very pendulous. When they are very
large, they can interfere with normal daily activities and have to be removed for this
reason. The large ones are call plexiform neurofibromas and they can have
subcutaneous nerve involvement so physician must be cautious in removal.
the lesions themselves are not malignant but there can be malignant degeneration of
these lesions because they are composed of neural tissue
Other findings of neurofibromatosis

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can also have caf au lait spots (the same finding that we find in tuberous
sclerosis)- light brown colored oval macules. 6 or more with a diameter greater
then or equal to 1.5cm is known as Crowes Sign.
o Axillary freckling these are freckles in the axilla, and are quite specific for
neurofibromatosis. Usually freckles occur on sun-exposed area so this is
unusual to find them there.
Normal intellect. There is a small percentage of patients in which there is some mental
retardation, and they can also have seizure disorders
This condition is associated with these same types of tumors that occur internally. And
they can affect different organs like the kidney and the heart. Pheochromocytoma may
be present.
Many people think that the elephant man had neurofibromatosis, but thats not true. The
man had something else, called Proteus Syndromebut he wont go into that, but it was
not neurofibromatosis
o

Peutz Jegers Syndrome

Characterized by pigmented macules on the mucosal surfaces (lips, buccal mucosa,
tongue, mouth, nose, palate, and fingertips)
The importance of Peutz Jegers Syndrome is that it is associated with polyps (polyposis)
of the small intestine. These polyps can cause intussusception in affected children.
These children are at an increased risk of developing malignancy in adults. However the
percentage of the malignant change in these polyps is quite smallonly 5% degenerate
into malignancy.
Hypermelonosis will fade if the polyps are removed. Thought to occur because the
tumor (polyps) may be secreting something similar to MSH (melanocyte stimulating
hormone).
SKIN DISEASES ASSOCIATED WITH INTERNAL MALIGNANCIES
(And the skin lesion itself is also malignant!!)
Bowens Disease
Its a rather nonspecific lesion. Its hyperpigmented
o In darker skinned races, it can be brown to light brown
o In lighter skinned races, it tends to be erythematous
It has somewhat irregular borders, and you can have quite a bit of scaling
Again, its very non-specific. A dermatologist might not even be able to make a clinical
diagnosis of Bowens disease. Its really a dx thats made by biopsy
Its an intraepithelial carcinoma (Carcinoma insitu). Dry scaly patches with some
crusting. Looks like eczema. But treatment with topical steroids does not work.
Can be associated with internal malignancies, too. Especially if its seen in covered
(Sun-protected) areas of the body. Associated with a number of malignancies, especially
of the lung and GI tract (most common). So make sure you examine the whole skin
surface of the patient.
Paget's disease
Youve heard of Pagets disease of the nipple in the previous lectures, and this is really
associated with intraductal carcinoma of the breast. It is usually unilateral.
This is extramammary Pagets disease. You dont see scaling, dryness and
erythematous eruptions. Usually hyperpigmented plaques. This is an area of the vulvar

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area. This can be associated with malignancies of the reproductive tract in women
(ovarian cancer, uterine cancer, or cervical cancer those types of things). Associated
with malignancy in the anatomical area where it is found.
In males it can be associated with Prostatic Carcinoma
Pagent cells would be found on biopsy
HIV DISEASE

Kaposis Sarcoma
Weve already heard of this, but here are some important points about the disease:
Classically was a lesion of elderly males of Mediterranean decent (patients who lived in
Italy, Greece, Israel, etc). Patients usually in their late 70s and 80s.
o Lesion was found on their lower extremities.
o This is a multicentric sarcoma, so you can have the lesions elsewhere as well as
on the skin. Areas that can be involved are liver and heart)the importance of
Kaposis is that we originally saw it in elderly males.
It is part of the AIDS complex now. We see this in younger males who are HIV positive
(at least in this hemisphere).
o Also. Its occurring not only on the lower extremities, but its occurring on other
parts of the body as well. Here we see a patient who has it on the hands.
o Traditionally it was plaques and nodules that you could see, but in the form
associated with AIDS, they are flatter more macular lesions.
o The other anatomical area affected in patients with Kaposis and AIDS is the GI
tract, so this can also be a marker for GI involvement. So patients with AIDS and
Kaposis need to be evaluated by the gastroenterologist and have endoscopy
done
This can actually be one of the causes of death in AIDS patients with
Kaposis sarcoma GI Hemorrhage
Picture: again this is a picture of the classic form of Kaposis sarcoma, involving the
lower extremities.
Picture: This is the type were seeing more often with AIDS patients. Remember its
flatter and not as elevated, nodular, or plaque-like as the traditional form was.
LYMPHOMAS AND LEUKEMIAS
Mycosis Fungoides
This is another malignancy of the skin, but its a cutaneous T cell lymphoma. We had
some discussion about this so he wont go into detail
Picture of an affected breast
Scaling and dryness
Starts internally and goes to the skin, or you can have the opposite scenario where it
starts in the skin as a localized lymphoma and then invades locally to the lymph nodes
and other organs
There is usually a history of long term chronic skin disease in these patientslike
eczema or ectopic dermatitis for many years (like 15-20 years). And then that becomes
mycosis fungoides so its thought to be due to chronic antigen stimulation of the skin.
However we do see a form in darker skinned races (African Americans, Hispanics, and
Asians), in which they present without a history of inflammatory skin disease (like
eczema or ectopic dermatitis. You dont see scaling or dryness in this form, you see

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hypopigmentation of the skin, which sometimes can be confused with vitiligo this is the
hypopigmented variant
Very often patient can have lymph node and bone marrow involvement, and what you
see is a Sezary cell this is a lymphocyte with a hyperchromatic, hyperconvoluded
nucleus that you see in the peripheral blood.

Leukemia Cutis
People with this lesion have chronic myelogenous leukemia.
In leukemia, you can see widespread infiltrative lesions of the skin. These lesions can be
plaques, they can be nodules as well. But if you biopsy the lesion you will see the
leukemia cells in the skin itself
Picture: This is another patient with leukemia cutis. Again you can see the high level of
infiltration of the skin. These plaques can merge with each other and almost become
continuous. This shows you how infiltrative leukemia can be in the skin in advanced
stages.
Differentiate from mycosis fungoides because its not dry-looking and no scaling.
Metastatic Carcinoma
There are a number of organs that are important here.
o In women remember breast and lung
o In men remember lung and colon
Spread of the tumor to the skin is a late process, implies poor prognosis, has usually
already spread to other organs. 5-10% of patients with malignancy will have metastasis
to the skin, which is a small number considering the large surface area of the skin.
Skin metastasis will be in the region of the primary tumor. Tumors are usually multiple
and hard, this is how you can tell it is metastatic. Can ulcerate. If you biopsy the tumor,
you will get the histopathology of the original tumor.
o I.e. a woman with breast cancer might metastasize to the chest or the upper
abdominal area.
o In men, the lung cancer will metastasize to the chest.
o Colon carcinoma metastasis will be to the abdominal area, particularly the
umbilical area. Theres a finding called the Sister Joseph Nodule (which we
learned about yesterday) in which there is metastasis directly to the umbilicus
from intestinal tumors that are malignant.
Another area that is quite common is the scalp. Metastasis to the scalp is usually
from Renal Carcinoma. This is very common because the scalp has such a large blood
supply, and the cancer can spread hematogenously. The majority of the cases of internal
metastasis to the skin will be solitary nodules. Its unusual to have widespread
metastases. If you have this, then its a very late process. Its already a late process
when it goes to the skin, but when its generalized, its a VERY late process.
There are a few exceptions to the nodular forms of presentation of the skin
o Metastasis on cruiasse specific for breast cancer
Inflammatory lesion of the skin (normally in metastasis, it will be noninflammatory).
Plaque-like lesion, as opposed to a solitary nodule that you see in most
cases of metastases.
This will most often go to the chest
o Multiple nodules normally it will be a single nodule in metastasis

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can be ulcerated
This one [referring to picture] is on abdomen, so the source was a colon
tumor
Pancreatic tumors also metastasize to the skin, but thats a very late stage.
Usually the diagnosis of the internal malignancy will be made prior to its skin
manifestations. But obviously the biopsy of the metastatic nodule will give you the same
pathology as the original malignancy thats internal.
Prostate cancer metastasizes quite often to the skin on the penis and the peri-anal area.

Sarcoidosis of the skin

These are often annular or ring shaped lesions that occur on the face.
They occur often on body openings like around the mouth, nose, and eyes.
The annular shaped lesion is also a common presentation in secondary syphilis which is
considered the great imitator with multiple cutaneous manifestations. Sarcoidosis is the
second great imitator on the skin.
When you biopsy this, you will see the characteristic noncaseating granulomas that are
typical of sarcoidosis.
We see a lot of cutaneous sarcoidosis here because of our pigmented population. In
African Americans, we can see this type of sarcoidosis in which you have
hypopigmentation around the sarcoidal granulomas. This is more common in darker
skinned races
When you have sarcoidosis in the skin, you can be limited to only the skin. But you can
make the diagnosis of systemic sarcoidosis from the skin biopsy.
o When you have a patient that you diagnose histologically on the skin, you do
send them for a pulmonary workup. But there are some cases where its only
limited to the skin. This is unusual, but it can occur.
Skin lesions are usually the first manifestations. Usually they will biopsy the lesions and
then send the patient to pulmonary for further workup.
So essentially when the patient is worked up and found to have the internal sarcoidosis,
whatever treatment that is used for internal involvement will usually help the skin to
respond as well.
o Systemic steroids should not be given only for skin involvement. That should only
be used systemic involvement of sarcoidosis
o There are other drugs that we use for cutaneous involvement if the person does
not have internal involvement, and these include topical steroids for localized
involvement of the skin, and if there is diffuse involvement of the skin without
internal involvement, you can put them on antimalarial drugs such as Plaquenil
(AKA Hydroxychloroquine Sulfate for you scholars out there )
SKIN MANIFESTATIONS OF CONNECTIVE TISSUE DISEASES
He wont go into all the systemic manifestations of the diseases. Only the parts pertaining to the
skin.
Systemic Lupus Erythmatosis
Butterfly rash (Malar eruption) goes across the malar area and across the bridge of
the nose as well.
o You can have skip areas so that the entire malar area is not involved. So this can
sometimes be confusing.

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This can involve malar area and can also involve the chin, upper lip, and the
cheeks.
o Whenever you talk about the malar eruption or the butterfly rash, its systemic
lupus!
Periungual telangiectasias Another manifestation of lupus, but generally of all
connective tissue diseases.
o You will see these dilated blood vessels, these telangiectatic areas in the
periungual area.
o So the next time you have a patient with connective tissue disease (regardless if
its lupus, scleroderma, etc), take a look at the periungual areas and you will see
this
o Its not specific for lupus, but it can be found in lupus
Alopecia (hair loss) this is the scarring type (sycotricial) of alopecia so its permanent.
o You can have localized areas (on biopsy of the skin, you will see complete
absence of follicles and dense inflammatory lymphocytic infiltratethis means
that you have deep scarring and that the hair will not grow back there)
o Or you can have diffuse alopecia (which is what you usually see), which is
thinning of the hair
o Alopecia is one of the diagnostic criteria for lupus according to the American
Rheumatologic Association.
Photosensitivity to sunlight or artificial light itchy eruption on sun exposed areas (.e.
forearm and the V of the neckin the picture, these were the uncovered areas. The rest
were not exposed) this is another diagnostic criterion by the ARA for diagnosis of
systemic lupus.
o

Discoid Lupus
This is another form of lupus, and it affects ONLY the skin. Most patients with DLE will
not have systemic manifestation of the disease. Those with high titers should be
followed.
Atrophy (depression) on the lesion. Burnt-out lesions- can be very disfiguringm almost
looks like a cigarette burn.
Will very often affect the ear (outer ear).
There is skin involvement only, but a certain percentage (about 10%) of patients with
discoid will progress to SLE so you still do a workup of these patients who have
discoid lupus. You would do immune titers on a yearly basis, and sometimes you can tell
by a change in the titer whether the disease has progressed to systemic lupus.
Morphea
Localized Scleroderma
Usually a hypopigmented, firm patch of skin in the affected area
Lupus Profundus
This is another specific form of lupus.
This is involvement of subcutaneous fat with a dense lymphocytic infiltrate.
Because subcutaneous fat is involved, these lesions are very painful. So if there is
pressure there, or if youre examining the patient, it will cause them quite a bit of pain.
Areas with more adipose tissue will be affected (like the abdominal area and the
buttocks)

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This is more of an inflammatory lesion, and you can see that there is marked erythema
there.

Scleroderma
You can tell this is scleroderma, with the very tight bound down skin giving shiny
appearance.
Patients will have a lack of facial expression because they cant wrinkle their forehead
Lips have a purse-like appearance, and they have mouse-like facies (very bound down
skin and purse-like lips)
this pattern is seen in all racial groups
Mat telangiectasias (telangiectasias on the face) mat means flatthis is specific
for scleroderma!
Sclerodactyly You can see that the bound down skin affects the mobility of the joints
and causes deformity of the joints. Patient will have no mobility in the hands when this
happens.
Calcinosis cutis (calcinosis of the skin) which can lead to ulceration
One subset is CREST Syndrome you can see that a good portion of this syndrome is
surrounding the skin findings You would think this is a bad thing, but actually CREST
syndrome patients have a better prognosis (they live longer than non-CREST patients)
o Calcinosis cutis
o Reynauds phenomenon
o Esophageal dysmotility
o Sclerodactyly
o Telangiectasias (Mat telangiectasias)
Dermatomyositis
Heliotrope eruption dusky erythema that occurs on the upper half of the face. Its
dusky because its not a bright erythema. It has more of a subdued, almost purplish color
to it.
o often affects the eyelids
Associated with myositiswont go into this portion of the disease
other manifestations
o Periungual telangiectasias (also with lupus)- telangiectasias under the
fingernails. Not pathoneumonic for one connective tissue disease, but is seen in
SLE and Scleroderma
o Gottrons papules hyperkeratotic papules on the knucklesfound specifically
in dermatomyositis

Rheumatoid Nodules
Often on the elbows, hands, fingers. Seen in 20-30% of patients with rheumatoid
arthritis
These are mucinous deposits in skin. These are subcutaneous nodules. They do not
orginate from bone, but occur at boney appendages.
Picture: rheumatoid nodule, and on the side you can see pyoderma gangrenosum
(ulcerations on the skin. You get a purple border that is underlined). This can be seen

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with rheumatoid nodules, inflammatory bowel disease, Leukemia, Polycythemia Vera,
and initially in HIV.
HYPERSENSITIVITY SYNDROMES
Morbilliform eruption
Picture: This patient had abdominal surgery (as you can see from the scar from the
linear incision and the sutures). And then he developed an erythematous (what we call
morbilliform) eruption. The reason we call it morbilliform is because it looks like measles
(it has the same morphology as measles).
The reason this is a hypersensitivity reaction is because its a morbilliform drug eruption.
This patient was put on antibiotics after abdominal surgery and had an allergy to the
antibiotics.
This will be the most common type of drug eruption you will see in hospitalized patients.
Treatment: stop the offending agent and the eruption will disappear.
Erythema Multiforme
This is a common hypersensitivity reaction
Characterized by round target-like lesions (AKA bulls-eye or iris lesions) concentric
rings
Common area is on palms
Can have oral lesions mucosal lesions look similar morphologically
This is usually due to drugs (these drugs include antibiotics and oral contraceptives that
have estrogens and progesterones, but its the estrogen that they think is more
responsible for erythema multiforme)
Can also be associated with some more unusual things like:
o mycoplasma pneumonia
o chronic herpes simplex infection.
The patient starts with a subclinical infection that may not be apparent
clinically, but the reaction they are getting on the skin is erythema
multiforme
So as to not confuse you, when it occurs with herpes simplex, these
lesions are not infected with the virusIt is just a manifestation of
hypersensitivity to the virus
Stevens Johnson Syndrome
Erythema multiforme can go on to Stevens Johnson Syndrome. This is a more severe
hypersensitivity reaction in which you have hemorrhagic crusting of the mouth, you can
see it in the conjunctiva, and you can also have involvement of genital mucosa
Sometimes it can occur de novo. It does not have to occur as erythema multiforme first.
The hypersensitivity is usually due to drugs
Treat this with high doses of Prednisone
Toxic Epidermal Necrolysis
This is the WORST type of hypersensitivity reaction
Patients have extensive areas of exfoliation of the skin. [Picture]: you have a patient
starting out with it where the skin has become completely denuded. The epithelial layer
just comes off completely

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o [Picture]: This patient has more extensive toxic epidermal necrolysis.

This is a life threatening condition.
Patients essentially have severe fluid and electrolyte imbalance, similar to patients who
have third degree burnsso these patients will have to be admitted to a burn unit or an
ICU for intensive management.
The mortality rate of this condition, if left untreated is relatively high (approaches 50%)
Historically, we used to treat them with high doses of steroids (close to 200mg of
Prednisone a day), but studies have shown that this is actually detrimental, so the
current treatment is IV-Ig
Due to inhibition of fas-ligand (he wont go into the mechanism)

Erythema Nodosum
Characterized by painful tender erythematous nodules on anterior tibial surfaces
Occurs primarily in women
This is a panniculitis (inflammation of the subcutaneous fat), which is very similar to the
lupus profundus that he mentioned earlier. Because you have inflammation of the
subcutaneous fat, it is very painful.
There can be a number of other causes
o Drugs Antibiotics, Oral contraceptives
o Manifestation of systemic diseases
Sarcoidosis so patient could present with erythema nodosum, and they
would need to be worked up for sarcoidosis)
Deep fungal infections including histoplasmosis and Cryptococcosis
Bullous Pemphigoid
You have tense bulla on an erythematous base
This is very often idiopathic. We see it in the elderly
It is not life-threatening in most cases, but it can be a hypersensitivity reaction to drugs
This is contrasted to pemphigous vulgaris
Pemphigus Vulgaris
You have flaccid bullae (compare to the tense bullae of bullous pemphigoid)
We can also have extensive exfoliation of the skinand they have septicemia due to
infection of the skin.
One of the main causes of death in these patients is septicemia. (hence its life
threatening)
So the difference between the 2 is that bullous pemphigoid is NOT life threatening and
pemphigus vulgaris is life threatening
Immunofluorescence
indirect
o done on patients serum (looking for antibodies). In a number of the skin
diseases that we have, the patients will produce antibodies to the skin (to specific
keratins or to other specific antigenic determinants in the skin). So we can detect
those by immunofluorescence.

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o
o

o
o

Take patients peripheral blood and incubate the leukocytes with immunoglobulin
and then they are attached to a fluorecine tag.
When you look at this under the immunofluorescence microscope, there is a
specific fluorescence that will occur and you will see the specific fluorescence by
the specific antibodies that youve used to label it with (because you use different
antibodies for different components).
You can tell which antibodies that patient has circulating in their skin against a
certain component of the skin. That way you can make the diagnosis without
having to do a skin biopsy.
The importance of the indirect immunofluorescence is that you can follow the
antibodies with the treatments. (i.e. A patient with pemphigus vulgarisif you
treat them with the appropriate drugs like hydrosteroids or cyclophosphamide,
you can see the titers dropping)

direct
o You take a skin biopsy of affected area, but well do the same thing. Well label it
with monoclonal antibodies and then tag it with fluorecine then well look at the
skin specimen itself under the immunofluorescent microscope (looking for
immunoreactants like Ig, Complement, etc). Do the same thing as before.
o [Picture]: This is what we see on direct immunofluorescence, and this happens to
be a patient with lupus. This patient has IgG, IgM, and C3 that are deposited
along the basement membrane zone (dont need to know all that)
o Again we know which antibodies weve labeled, and with the
immunofluorescence we can say that those specific immunoreactants are found
at the dermo-epidermal junction in this patientand that is how we can make the
diagnosis of lupus.
o We can make the diagnosis of a number of the other immunologic mediated skin
diseases by this same technique Bullous pemphigoid, dermatitis herpetiformis,
pemphigoid vulgaris, and others that we dont need to know
There is a specific test for lupus. When we use the direct immunofluorescence for lupus,
we see this positive fluorescence, we call it a positive Lupus Band test.
o The importance of this is that it gives you a prognosis of the patient. Take the
skin specimen from a covered area (a non sun exposed area like the buttocks)
if you see positive Lupus Band Test (i.e. positive immunoreactants) in this area,
thats a poorer prognosis and they probably have renal involvement.