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Marsha Gordon, MD
Bullous (Blistering) Diseases
* Pemphigus vegetans is a rare variant of pemphigus vulgaris. Pemphigus
vulgaris is an autoimmune disease of the skin. Patients with pemphigus vulgaris
have auto-antibodies that respond to antigens that are found in the epidermis
(top layer) of the skin. These antibodies are found in the intercellular spaces
between epidermal cells.
* When the antibodies attach to the antigens, the skin can no longer stay intact,
and it breaks apart and blisters up.
* Pemphigus vulgaris is a blistering disease, but most of the skin will be denuded.
The blisters are so superficial that by the time the patient presents to the
physician you rarely see intact blisters. If you do see intact blisters, they are very
fragile and easily breakable.
* This disease affects the skin and the oral mucosa. Erosions in the oral mucosa
may occur before skin is involved. The disease was fatal prior to discovering
corticosteroids, now we treat with high dose prednisone and steroid sparing
medications like azathioprine.
* Many disease present looking similar to pemphigus vulgaris. If you see a slide
with blisters and denuded areas, think pemphigus but realize the actual
diagnosis is made by biopsy.
* Biopsy must show the epidermis breaking apart one cell from the other, and
also immunofluorescence can show the antibodies being deposited in little circles
around the epidermal cells.
* Pemphigus foliaceus and pemphigus erythematosus are more superficial forms
of pemphigus. They almost never present with intact blisters.
* Bullous pemphigoid is an autoimmune disease of the skin with antibodies
reacting against antigens at the dermoepidermal junction zone (DEJZ). They do
not react with anything in the epidermis per say, they react at the DEJZ. As a
result, the blisters are deeper.
* There will be intact, tense, blisters with some denuded/eroded areas. The
blisters are tougher, tend to be tense, and often are intact when they present.
* Bullous pemphigoid presents in an older population. Pemphigus presents
classically around ages 30-40. Bullous pemphigoid is seen in a geriatric
population classically, ages around 70-80. This does not generally involve the
oral mucosa, a differentiating feature between the two.
* Bullous pemphigoid biopsy will show blister with entire epidermis lifting and
separating from the dermis. Immunofluorescent will show lighting-up, a linear
line, at the DE junction.
* Pemphigus and pemphigoid tend to heal well with treatment, steroids.
* Cicatricial pemphigoid is a less important variant and does scar even with
treatment. It almost exclusively involves the mouth and eyes; so scarred tongue,
scars around mouth and blindness from eye involvement.
* To make a TEN diagnosis, we do a biopsy and send it for frozen section because
we want an answer quickly.
* On frozen section, we see complete necrosis of the entire epidermis. The entire
thickness of the epidermis is lifted and separated from the dermis and is
necrotic/dead. In SSS, the break is way high up in the epidermis.
* TEN treatment is stop medications, put in laminar flow room to prevent
infection, watch fluid and electrolytes, steroids are highly controversial because
they mask infection, which is a major cause of death in TEN. Because eye
involvement can occur, must have an ophthalmologist involved in this care.
* Fixed drug eruption refers to a situation when a patient is exposed to a drug
they are allergic to. In one are, they develop either a blister or a red mark. If you
take away the drug, it heals. If you give the drug back, in the exact same
location, the same reaction occurs. We do not know why this happens, it is just a
curiosity. It is benign.
* Fixed drug eruption generally heals with a dark mark that can take a long time
to fade.
--------------------------------------------------------------------------------------------------Bacterial Skin Infections
* Tinea pedis (fungal infection of foot) caused by dermatophytes (fungus that
attacks the skin). Tinea pedis classically involves the interdigital webs between
the toes. There is a blistering form of tinea pedis, bullous tinea pedis, usually
involving the soles of the foot. The blisters are intact and if you open and do a
KOH prep you will see it teeming with dermatophytes.
* Fungal infection of the toenails is onychomycosis. Tinea cruris is fungal
infection of the groin. Classic pattern is annular (round), red, scaly border,
extending over time.
* Tinea capitus usually seen in kids with a patch of hair loss (alopecia) or
thinning. Sometimes the skin is scaly and peeling. Sometimes there will be a few
little pustules, pus bumps. Tinea capitus can scar if not treated, giving a
permanent scarring alopecia.
* Treatment of tinea capitus is oral anti-fungals. The infection can get down into
the roots so topicals will not reach.
* Tinea corporis can be very subtle with a tiny pink scaly area. Many times you
will think this is just eczema, but dont forget it could be a tinea corporis. If you
cant tell the difference, do a culture (takes weeks) or a KOH prep.
* Honey colored crust, think impetigo. Caused by staph or group A strep
(pyogenes).
* Certain strains of staph exude a toxin, exfoliatin. This toxin can cause the skin
to blister. Say patient has impetigo that is caused by one of these types of staph
(bullous impetigo), you will see the honey-colored crust and blisters around. The
blisters have purulent material in them and are teeming with staph.
* Treatments involve covering for staph and for strep.
* Think about any other bacteremia here also, like staph bacteremia. Not
everything that is purpuric is not a major infection. Senile purpura is seen in
elderly patients with fragile blood vessels and connective tissue. They can get
large purpuric areas with very minor trauma.
--------------------------------------------------------------------------------------------------Pigmented Lesions
* Pigmented lesions come in all shapes and sizes. Keep in mind symmetry. Is this
lesion symmetrical? Yea, look at ABCD, border, color, diameter smaller than
pencil eraser, but symmetry matters. Benign pigmented lesions tend to be
symmetric. Once a malignant pigmented lesion has occurred, the growth is out
of control.
* A junction lesion, symmetric, is a benign lesion with the melanocytes found at
junction (epidermis/dermis).
* Compound nevus, benign, symmetric. Some of the nevocytes are at DE
junction and some down into dermis.
* Dermal (intradermal) nevus is skin colored, symmetrical, benign.
* Halo nevus is a symmetrical halo of lightness around a nevus. This is benign.
Lymphocytes are destroying nevus.
* Nevus with irregular border, irregular color, this is melanoma. Melanomas start
with melanoma in situ, where it is only present at the epidermis. If it presents at
this stage without dermis involvement there is 100% cure with proper excision.
Melanomas are asymmetrical and irregular.
* Nodular melanoma may be more symmetrical than others, but it is still
irregular.
* Most common melanoma is superficial spreading type, 70%. This has an in situ
phase lasing 6months to a year. Next most common is nodular, 15% of
melanomas. It appears and immediately invades. Other types are acral
melanoma (hand, feet) with high mortality and lentigo malignant type seen on
the sun exposed areas of elderly
people and has good prognosis.
* Stages are I (skin), II (lymph), and III (mets). Prognosis is based on level of
depth of invasion.
* Greasy stuck-on appearance is seborrheic keratoses, overgrowth of top layer of
skin, benign.
* Actinic keratoses refer to an area of the skin which is scaly and caused by sun
(sun exposed area). They are precancerous, but the majority of patients do not
develop cancer. Can turn into squamous cell carcinomas.
* 75% of squamous cell carcinomas on the skin are sun-induced. They have a low
metastatic rate, around 1%. If we can find them and treat quickly, we can cure
the patient in most cases. The exception is squamous cell carcinoma of the
mucosa (lip) where there is a metastatic rate 10-20%.
* Basal cell carcinoma has pearly raised border with crust, broken blood vessel
on it possibly, most common of all cancers. Generally do not metastasize. Should
be removed.
* Kaposi sarcoma seen in immunosuppressed population (AIDS,
lymphoproliferative, chemotherapy) and elderly (Mediterranean). Sarcoma is a
misnomer, probably a neoplasm of epithelial cells likely lymphatic and small
blood
vessels. Bluish plaque like discoloration. Diagnosis with biopsy.
--------------------------------------------------------------------------------------------------Papulosquamous (Scaly) Eruptions
* Psoriasis is silvery scale on a red base. Usually involves the knees and elbows,
rarely the palms, sometimes the scalp. Can cause nail changes (pits) and
generally nail dystrophy (onycholysis).
* Eczema (dermatitis) can be asteatotic, meaning eczema simply on the basis of
dryness. Skin gets so dry that is simply breaks down.
* Atopic (allergic) eczema is usually seen in atopic families. Patients will have
asthma, hay fever, and eczema. They have lots of IgE in system, specifically to
staph. So they react in an expected way to the staph that is on our skin, with red
itchy patches in the flexures. Skin can be thickened from scratching. Ask about
family atopy.
* Treat by removing things that cause allergy.
* Seborrheic eczema is a chronic condition with redness and scaling in classic
locations, not well understood.
Locations are the nasal labial folds, eyebrows, around ears, scalp.
* Stasis dermatitis begins with venous insufficiency. Formed elements in blood
cells like hemosiderin leave the dark spots. Protein surrounds the vessels causing
fibrin cuffs and preventing oxygen exchange so tissue becomes hypoxic and
breaks down.
* Contact dermatitis from irritation or from allergic contact dermatitis. In allergic
contact dermatitis, you see well demarcated rash only at location of problem
(e.g. poison ivy in linear blisters).
* Pityriasis rosea looks like secondary syphilis, oval slightly scaly salmon-colored
patches over the body. It never involves the palms. It classically begins with a
herald patch. Get a VDRL to rule-out syphilis also.
* Decubitus ulcers are defined in stages. Stage I is non-blanching redness. Stage
II is very superficial breakdown. Stage III is full-thickness skin breakdown but not
to fascia. Stage IV means all the way through to bone or muscle. Decubitus
ulcers caused by pressure, sheering forces (slipping down in bed), friction,
rubbing, moisture from incontinence (urinary or fecal).
--------------------------------------------------------------------------------------------------Nail & Hair Disorders
* Acute paronychia is an infection, usually staph, in the area around the nail.
Seen often in patients who get manicures. Sometimes you see a pus bump,
sometimes just red and tender. Generally culture but always cover for staph.
Treat quickly because if swelling is great enough you can impair the blood supply
to the distal tip of the finger. So decompress, drain, soak, treat with antibiotics.
* Clubbing refers to a distal bulbous enlargement of the finger tip. It is
sometimes seen in a familial setting but most often in a patient with chronic lung
problems, such as emphysema, bronchiectasis, tuberculosis.
* Clubbing can be seen in cyanotic heart disease as well.
* Hypertrophic osteoarthropathy looks like clubbing except there is tenderness at
the distal fingers. There will be periosteal thickening of the distal phalanx and
often gynecomastia. Associated with lung carcinoma.
* Alopecia areata is oval or round patches of complete hair loss. Alopecia areata
totalis covers all the hair in the head, universalis covers all the hair of the body. It
is believed to be an autoimmune type of disease but poorly understood. It can
also involve the nails, with nail dystrophy.
* Traction alopecia comes from chronic pulling of the hair so much so that the
roots are destroyed. Seen in patients who wear tight braids or tight pulling. This
is a permanent scarring alopecia.
* Telogen effluvium seen after patient goes through tremendous physiologic
stress, surgery, delivery of a baby, infection, sometimes many of the hairs will all
go into a resting phase (telogen). The body is stressed so it is shutting off nonessential functions like hair growth. When new hairs begin to grow in a month or
two, the new hair pushes out the old hairs and there can be an enormous
amount of hair loss. These hairs will grow back, but there will be a few months of
hair loss. The history makes the diagnosis here.
* Androgenetic alopecia is male-pattern or female-pattern baldness. Often begins
in 20s-30s and continues throughout life.
--------------------------------------------------------------------------------------------------Benign Skin Growths
* Gout is caused by chronic elevated uric acid levels. Uric acid begins to deposit
in the joint, causing joint pain. Over time it will deposit in skin giving chalky
subcutaneous masses called tophi. Tophi is sodium urate crystals deposited in
the skin. They are benign.
* Acrochordons (skin tags) are benign and not associated with internal
malignancy or anything else. Could be a personal or familial tendency. Seen
more frequently in obese patients possibly more frequently in diabetics.
* Sebaceous cysts divided into epidermoid cyst and pilar (trichilemmal) cyst. It
depends on where the cyst originates from, if from dermis it is epidermoid and if
hair follicle then pilar cyst. Either way, skin epithelium or hair follicle epithelium
invaginates under the skin and makes a pocket. That skin keeps making
oil/sebum but is in a pocket. This is completely benign. If there is no punctum
connecting it to the surface, all you see is a nodule.
* 90% of cysts on scalp are pilar cysts but you really cant tell until you remove
them.
* Digital mucus cyst are soft, could be a collection of mucopolysaccharides
(benign and we drain) or a herniation of a joint sac. Either way, no need to worry.
* Hemangiomas are benign collections of blood vessels. There are two types,
capillary and cavernous. Capillary hemangiomas also called strawberry
hemangiomas, generally appear early in life and enlarge during first year of life.
By the end of the first year they generally stop enlarging and involute. They
involute each year such that by the time the child is 9, 90% have completely
disappeared.
* Cavernous hemangioma appears in infancy and persists, it grows with the
patients. It is due to deeper dermal vessels. Also benign. Biopsy of this is difficult
and causes lots of bleeding, so dont do it.