Click here to view next page of this article


Cutaneous Manifestations of Systemic Diseases

Erythema multiforme is a classic disorder induced by many drugs. You see the classic hemorrhagic kind of erythema that can take place and here you see the individual lesions. Some of them are almost starting to become bullous. The classic iris lesion is characteristic. Unfortunately, not all patients look like this and we get variations of these expressions which make it extremely difficult. So you have to evaluate everything you see and the symptoms of the patient and look at the overall picture before you can arrive at a erythema multiforme.

Erythema nodosum, which are these nodules of usually the anterior tibia. They are very painful and the specific cause is only found in a very small percentage of the patients. I think it is even less than 50% which is usually seen in the literature. Drugs are notorious but many disorders cause this disorder. When one sees these patients come in with these extremely painful nodules on the anterior tibia, they resolve sometimes with just symptomatic care within three to six weeks. Elimination, for instance, with a drug, they will disappear within three to six week.

Lyme disease eruptions and here is a classic example of an annulare erythema precipitated by the Arthropod bite. Here it is again at an older stage and here is one, of course, where it is most difficult to decide what this eruption is but it turns out thatís what it was.

Systemic lupus erythematosus is really an enormous spectrum, as you all know. Iíve listed some of the characteristic classifications of the clinical forms for you and they are not easy. Especially each one of these has variations that will drive you to the wall but here is a classic example of a patient, a young lady, many years ago of mine who had acute systemic lupus erythematosus and went on to die.

Dermatomyositis is associated with heliotrope eyelids, which are rather characteristic. If you look at not just the eruption but where the eruption is, this gives you clues. Here is a perfect example of that kind of a patient where the eruption around the eye is quite characteristic for this disorder. On the neck, the eruption isnít so characteristic. I would have real difficulty distinguishing this kind of

Scleroderma. All of us can make that diagnosis now when she is in the end stages of her disorder. But when you get an early scleroderma, it is extremely difficult to make the diagnosis, and you will rely on other kinds of laboratory examinations to do it. But 

Purpura is obvious. You put your fingers on the skin and press and it does not blanch. So you know you have a hemorrhagic phenomenon taking place in the vascular capillaries of the skin. One has to work the patient up for the obvious bleeding disorders and a general examination is indicated. We see this, of course, in many very severe diseases like Rocky Mountain Spotted Fever, meningococcemia, scurvy, and we it see in leukocytoclastic vasculitides. So we have to work the patient up generally when we see purpura. There are a few benign disorders such as Schambergís purpura which is a pigmented purpura which no one knows what causes it. Here is another example of a type of purpura and Schambergís purpura usually is from the 

Necrobiosis lipoidica diabeticorum is a cutaneous finding that  is fairly diagnostic. It sometimes even precedes the diabetes and precedes it by a long period of time. So it will be diagnostic that the patient is going to get diabetes. You see this 

Porphyria cutanea tarda is one of the porphyric metabolic disorders. Very common in the United States. How do you pick this up? The patient comes in complaining of easy bruisability of the skin of his hands. The exposed areas. Why the exposed areas? Because the patient has in his blood large amounts of urinary coproporphyrin which are photosensitizers in large quantities. The end result is that

Xanthomas or xanthelasma. Most of these, xanthelasma is not associated with some changes in the lipids but some of them are and so one has to work up these patients. Then we can get eruptive xanthomas as you see here in patients who have diabetes, for instance,

Neurofibromatosis and is a classic example of neurofibromatosis. One would have difficulty missing this patient. It is very obvious but

Adenoma sebaceum and tuberous sclerosis of Bourneville. Itís a congenital disorder and they develop these adenomatous lesions

Hereditary cancer disorders and show you how one can make a diagnosis and pick up from family histories hereditary disorders very easily. What are some of the characteristics of the family history of hereditary cancers? They usually have an early age of onset.

Muir-Torreís syndrome which is composed of sebaceous adenomas and sebaceous carcinomas and sometimes multiple keratoacanthomas. These patients have a prolonged survival even though they get some systemic cancers. This disorder has been

We started looking at family trees of patients who had HNPCC and we discovered that within these families, there were patients who had classic descriptions of Muir-Torreís syndrome with these sebaceous carcinomas and multiple keratoacanthomas. It turns out that this phenotypic expression of the hereditary nonpolyposis colorectal cancer families is an unusual phenotype. It is not found very often so weíve connected that to the syndrome. Indeed, people now doing genetic studies have verified our family histories that we took that connected these people with the Muir-Torres syndrome to the HNPCC. Not all HNPCC families show the phenotype of the

Melanoma, of course, is one that a dermatologist would always get interested in. Here I show you this slide to show you that most of the melanomas are sporadic but a good percentage of them are hereditary. Why is it important to separate these out and take a family

Melanoma is increasing at an enormous rate in this country. By the year 2000, I think the projection is 1 in 75, and there are even people who think now that the use of sunscreens has promoted this. There is some animal data to show that UVA light that passes

Familial atypical multiple mole melanoma or FAMMM syndrome. Thereís an abbreviation used by the Eastern group now after many changes, recently called the "familial atypical mole and melanoma syndrome". These patients have multiple funny looking moles. Theyíre big. Theyíre bigger then pencil erasers on their body. They also have them in unusual locations: the scalp, the buttocks or the dorsum of

They also have pigmented nevi on the iris, multiple ones sometimes and when they get malignant melanoma, they especially get multiple primary. We have one man who has 16 melanomas of the skin over a period of about 30 years and four of them were Clark level 4s and heís still alive. Remember the survival I talked about? So thatís important. In addition, in some families, they have multiple organs

This person didnít have a lot of moles but he had these big atypical moles and here is one on the scalp. This young man came from a family that had the FAMMM syndrome and he, on examination, didnít have many moles on his body. But I looked at his head and hereís this huge lesion, over a centimeter, on his scalp. We decided to take it out because thatís that unusual spot I talked about. Itís

What is the extent of hereditary cancers? Itís getting to be a lot more prevalent than we once thought they were. They are not as rare as they used to be thought. They may be as much as 10% of the patients, so itís important to identify these people because surveillance means that you can pick up the cancers early in these individuals. There are at least that many hereditary cancer syndromes, however. A gene is being discovered almost every day but some of them are the same gene with different variations. I think the one in the APC