Dermatology with Dr. Marsha Gordon, MDBullous (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.---------------------------------------------------------------------------------------------------Porphyria Cutanea Tarda* All porphyrias relate to abnormalities of heme metabolism.* Porphyria cutanea tarda (PCT) is the classic dermatological porphyria. The metabolites that accumulate are photosensitizing. Skin that is exposed to the sun, classically the dorsum of the hands, develops a photosensitivity.* Clinically we see blisters and erosions. This condition does not heal well; it heals with scarring and heals slowly.* The face also can be involved. The top of the scalp is another location.* Two other skin manifestations besides blisters. First, patients develop hyperpigmentation; they look like they are getting a tan. Second, they develop hypertrichosis, they become hairier. The classic place the hair is seen is at the tops of the cheeks.* Precipitating factors for PCT is alcohol, so middle-aged alcoholic with blisters. Estrogens are a classic precipitant, so women who are on birth control pills or hormone replacement. Iron may be a precipitant, and fungicide hexachlorobenzene may be a precipitant.* Treatment is to remove precipitating factor and use anti-malarial medication chloroquine. We also treat with blood-letting (phlebotomy) to try to drain off excess iron. It is a slow improvement and may take up to a year.---------------------------------------------------------------------------------------------------Hypersensitivity Reactions* Urticaria is the classic type I hypersensitivity eruption. It is mast-cell induced. Mast-cells have IgE antibodies affixed to them, and when proper allergen comes along, it attaches to IgE antibody on the mast cell and causes release of the mast cell package, including lots of histamine and mediators.* You see vasodilatation, causing hive pinkness. You see leakiness of the vessels, causing hive swelling.* These are immediate reactions, meaning if the patient is allergic to penicillin and you give them penicillin, you will get an urticarial reaction probably within 30 minutes to an hour at the most.* Any individual hive will last up to 24 hours. However, new hives will continue to present over weeks as long as the antigens are present in the system. This is important because other diseases can look like hives. If the urticarial hive lasts less than 24 hours then it is urticaria.* Morbilliform eruption is a faint pink morbilliform (measles-like) eruption. It may be slightly raised. This is believed to be a type IV hypersensitivity eruption, lymphocyte mediated.* If a patient is given an antibiotic and are doomed to develop a morbilliform rash, the first time they are exposed they will not develop a rash from somewhere between 10days to 2weeks. It is believed that this is the time needed to recruit and sensitize the lymphocytes.* The second exposure will result in a reaction within 3-4 days because they have been sensitized.* Morbilliform drug rash often seen with antibiotics.* Spectrum of hypersensitivity reactions begin with erythema multiforme, then to Stevens-Johnson, and finally to toxic epidermal necrolysis (TEN).* Erythema multiforme refers to target-like lesions, classically seen on the palms. However, they can be seen on other parts of the body. Classic example is a bull-shot with a dusky bluish color in the center and a red rim around.* The multiforme means it can take many forms. So there may be a blister in the center with a red rim around. Whatever the formation, it will be target-like, targetoid appearance. Classically involves the palms and soles.* If any mucus membrane involvement, the involvement has to be very minor to be called erythema multiforme.* Erythema multiforme often seen to drugs and also infection (herpes and mycoplasma pneumonia). Specifically with herpes, a recurrent infection, patient can get recurrent erythema multiforme with each outbreak.* Erythema multiforme is self-limiting, thus will go away if you remove the offending agent/disease.* Stevens-Johnson refers to more than minor mucus membrane involvement. Classically you see hemorrhagic crusts of the lips and oral mucosa. These patients will likely have skin involvement as well.* Stevens-Johnson has a mortality of 5-10% even with appropriate treatment, cortisone (prednisone).* Disease can last 2-3 weeks. With mucosal involvement, patient may not be eating well. So you see fluid and electrolyte abnormalities. With skin involved, patient is prone to infection.* Toxic epidermal necrolysis involves skin that is confluently red that burns or feel tender. Within a short period of time, the skin begins to bubble-up and literally sloughs off.* There can be 100% sloughing of skin, so like a 100% burn. These patients are tremendously ill. May have fever, elevated white count and elevated liver function tests.* TEN prone to electrolyte abnormalities and infection. Mortality is 25-50% even with appropriate treatment. This is almost always caused by a drug, phenytoin, barbiturates, carbamazepine, allopurinol, penicillins and sulfonamides. Think about this if called to a neuro management unit for a major skin disorder.* TEN can look very much like staphylococcal scalded skin (SSS) syndrome, but is treated very differently. Treatment of SSS with antibiotics, while TEN can be caused by antibiotics.* 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.* Staphylococcal scalded skin syndrome is from staph that exudes exfoliatin toxin, but the main site is distant from the skin (e.g. abdominal abscess). Say in addition the patient is pretty sick, poor kidneys or poor liver, older or very young. Because the patient is debilitated, they cannot break down this toxin and it spread throughout the body.* Exfoliatin toxin causes skin to exfoliate. It may be difficult to distinguish at the bedside from TEN because you see the skin sloughing off. If you culture or Gram-stain skin, you wont see anything because the staph is at a distant site and youre getting the result of the toxin.* Here you do a frozen section to differentiate from TEN. Here in SSS syndrome, you see intact epidermis expect for very superficial blistering. Superficial at the granular layer of the skin.* Treatment for SSS syndrome is anti-staph antibiotics. If TEN, then take them off antibiotics.---------------------------------------------------------------------------------------------------Viral Skin Infections* Herpes is a viral infection that classically involves the mouth or genitalia, mostly because those are the common areas of inoculation. It can be seen in other areas including the eye.* Clinically you see groups of blisters on a red base, such as cold sores. The first case of a cold sore for a patient can be extremely angry if they have no immunity to it. Lips can be very swollen.* Herpes comes and goes, often arising in times of stress. Oral herpes can be brought out by sun as well.* Say you see many groups of blisters on a red base, extending down a dermatome. This is shingles from herpes zoster, a reactivation of latent varicella zoster virus (chickenpox). Generally zoster begins with pain. Patient may present to physician thinking theyre having a heart attack, or kidney stone, or sciatica. A day later, the blisters begin to develop.* If you see a zoster involving the ear, think about Ramsey Hunt, tinnitus, vertigo, CN VIII, facial palsy.* If trigeminal ophthalmic division is involved with herpes you need an ophthalmologist.* Herpes zoster is disseminated if more than 20 blister outside of a dermatome. This means there is hematogenous spread. This patient is not controlling their zoster infection. Admit patient to hospital and treat aggressively.* Patients need a good general workup for any herpes zoster a month after healing because they may be immunosuppressed for a variety of reasons, associated with lymphoproliferative diseases.* Warts caused by human papilloma virus, many types. Not much to say about warts.* Cellulitis usually caused by staph or beta-hemolytic strep. Presents with red, warm, tender plaque. Usually pretty evident. Mark with pen to determine if treatment is working or if cellulitis extending. Often we cannot do a culture so treat for what you think it is (staph or strep).* Necrotizing fasciitis (flesh eating disease) is a medical emergency. Can be caused by a mixed infection or a strep infection. Generally it starts with a cellulitis and quickly the organisms go deep and make it to fascial planes, where they can spread across the planes and cause damage very quickly. Strep can travel extremely fast, from ankle to thigh in a single day.* Treat necrotizing fasciitis with antibiotics and it absolutely requires surgical debridement. There is no way the antibiotic will get into the necrotic tissue at the fascia in time unless the area is completely and widely debrided.* If there is a question, we can do a biopsy and see strep very deep. But we must treat quickly.* Syphilis starts with a chancre (primary), hard, nontender classically, associated with nontender adenopathy. Average time of onset of chancre is 3-weeks after exposure, can be from 10-90 days. The average time when the blood test turns positive is 4-weeks. So if you see the patient and think it is a chancre, you need to do a dark field exam of the chancre looking for spirochetes, treponema pallidum.* Secondary syphilis appears 6-8 weeks after chancre, see oval salmon-colored patches over the body with palms and soles involved. At this point, the blood test should be positive.* HIV-positive patients can quickly develop tertiary syphilis with CNS changes. Some believe you need an LP.---------------------------------------------------------------------------------------------------Other Skin Infections* Scabies is caused by human mite, sarcoptes scabeii. It causes an extremely itchy bumpy area, such as penis and scrotum. Other places classically involved are breast, especially areola, axilla often involved, and the interdigital webs of the hands.* Furrows or linear burrows may be a clue. Only an average of 11 mites on entire body. You need to scrap a spot and look for eggs. Itchiness due to stool.* Meningococcemia present with a brief upper respiratory infection. Next stage is a fever and mental status changes. If you see fever and rash, think meningococcemia. Petechia are non-blanching purple areas of the skin. The lesions have a smudges look and given time they will become vesicular and necrotic with a slate-gray center.* You may not see signs of meningeal irritation. If can be so acute and fulminant that there isnt enough time.* Do Gram-stain of CSF looking for organisms, increased protein and decreased glucose.* 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 elderlypeople 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 bloodvessels. 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 non-essential 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.