The Lupus Book: A Guide for Patients and Their Families, Third Edition (13 page)

‘‘bridge’’ between discoid lupus and SLE.

3.
Chronic cutaneous lupus erythematosus
, also known as
discoid lupus erythematosus (DLE)
. About 15 percent of all lupus patients are classified as having DLE, but patients with SCLE or SLE may also have discoid

lesions.

The cutaneous features of lupus include
mucosal ulcerations
—sores in the mouth, nose, or vagina;
alopecia
—hair loss;
malar rash
—the butterfly rash on the cheeks;
discoid lesions
—thick, scarring, plaquelike rashes;
pigment
changes
—both loss of pigment and more pigment in different places;
urticaria
—hives or welts; and
cutaneous vascular
features involving the blood vessels. Included in this category are
Raynaud’s phenomenon
—when the fingertips turn red, white, and blue in reaction to cold temperatures;
livedo reticularis
—a red mottling or lacelike appearance under the skin;
purpura
—which appears like a bruise or black-and-blue mark;
cutaneous vasculitis
—breakdown of the skin due to inflammation of the superficial vessels, which can lead to
ulcers
or
gangrene
, a breakdown of the skin due to inflammation of the deep vessels or a blood clot.

There are other conditions, not usually seen in lupus patients, that involve the skin and require attention. One condition is
panniculitis
—which involves inflammation of the dermis of the skin.
Bullous lupus
, another condition, produces fluid-filled blisters or a rash similar to that of chickenpox. Complications from the use of steroids as a treatment for lupus can also induce skin damage such

as
ecchymoses
, or black-and-blue marks, as well as
skin atrophy
, which results in paper-thin skin.

DISCOID LUPUS ERYTHEMATOSUS

Chronic cutaneous (discoid) lupus erythematosus (DLE)
is commonly known as discoid lupus erythematosus. It is diagnosed when a patient with a discoid lupus rash (confirmed by skin biopsy) does not fulfull the American College of Rheumatology criteria for systemic lupus (see Chapter 2); 10 percent of all lupus

patients have DLE. Remember, discoid lesions may be a feature of SLE.

In the United States, 70 percent of patients with DLE are women and 75

percent are Caucasian, with the mean age of onset in the thirties. Discoid lesions appear on sun-exposed surfaces but, in rare cases, can also be found on non-sun-exposed areas. Such lesions generally do not itch. They appear as thick and scaly; under the microscope, one sees plugging of hair follicles, thickening of
Reactions of the Skin: Rashes and Discoid Lupus

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the epidermis, atrophy or thinning of the dermis (the part of the skin under the epidermis, which is the topmost layer of skin). Signs of inflammation are also

present.

Aching joints and other constitutional symptoms are found in 10 to 20 percent

of patients with DLE. Blood testing shows a positive ANA test in about half of

the cases; other autoantibodies are seen in less than 10 percent. Anemia may be observed in 20 percent of the patients, and a low white blood cell count in half.

Discoid lupus can appear similar to other skin lesions. For example, rosacea,

fungal infections, sarcoidosis, seborrhea, dermatomyositis, and a sun-sensitive rash called polymorphous light eruption can be ruled out by a simple skin biopsy and blood tests before diagnosing DLE.

Without treatment, discoid lesions may progress. After many years, some may

turn into skin cancer. ‘‘Localized DLE’’ was a term coined at the Mayo Clinic

in the 1930s to describe discoid lesions appearing only above the neck. They

rarely evolve into systemic lupus and are treated with antimalarial drugs or local remedies. ‘‘Generalized DLE’’ implies lesions above and below the neck. This

form has a 10 percent chance of developing into systemic lupus. Discoid lupus

is managed by avoiding the sun and by using sunscreens, antimalarial drugs,

and steroid creams. Sometimes steroid injections are helpful with these lesions.

In rare cases, severe resistant lesions may require antileprosy drugs, such as

thalidomide or Dapsone, oral corticosteroids, azathioprine, or nitrogen mustard ointment. See Part V of this book for a review of these treatments.

SUBACUTE CUTANEOUS LUPUS ERYTHEMATOSUS

Subacute cutaneous lupus erythematosus (SCLE) is a rash seen in about 9 per-

cent of lupus patients; 20 percent with SCLE also have lesions typical of discoid lupus. Unlike DLE, SCLE does not scar the skin. Under the microscope, the

inflammation is mild and diffuse, not thick and scaly. The lesions in SCLE, like those in systemic lupus, also do not usually itch. The rash may look similar to that of psoriasis.

Among SCLE patients, 70 percent are women and 85 percent are Caucasian.

The mean age of onset is in the early forties. Half of these patients fulfill the American College of Rheumatology criteria for systemic lupus. Among the

SCLE cases, 75 percent are sun-sensitive, 65 percent have joint aches, but less than 10 percent develop organ complications (in which the heart, lungs, kidneys, or liver are involved).

We know little about the causes of SCLE, but certain drugs such as thiazide

diuretics (e.g., Dyazide) have been known to bring it on. Two-thirds of SCLE

patients have positive ANA and 90 percent have anti-Ro (SSA) tests. Nearly all

have the HLA-DR3 marker.

The lesions of SCLE are notoriously resistant to the usual drug therapies.

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Where and How Can the Body Be Affected by Lupus?

Skin creams and antimalarials provide only modest results. But retinoid (Vitamin A) derivatives have been helpful and are important in the management of acute

SCLE.

CUTANEOUS FEATURES OF LUPUS

Mouth or Nose Sores (Mucosal Ulcerations)

Mouth sores are seen in 20 percent of patients with systemic or discoid lupus.

Occasionally, nose ulcerations are noted, which may result in nasal septal per-

foration. In rare cases, women may develop recurrent vaginal sores. Oral ulcers must be differentiated from herpes lesions or cold sores seen in lupus patients, especially those who are on steroids or are receiving chemotherapy. The sores

may be solitary or appear as crops of lesions. They may be found on the tongue

or any part of the mouth.

Oral ulcers look like lupus under the microscope. They are managed conser-

vatively with old-fashioned remedies such as buttermilk gargles or hydrogen

peroxide diluted in a few ounces of water, gargled and spit out several times a day. A local steroid, triamcinolone, which can be found in a dental gel (e.g.,

Kenalog in Orabase), can be applied to the lesions and usually brings about

prompt healing. Antimalarial drugs and systemic steroids are also helpful. Nasal ulcers sometimes respond to a petroleum jelly such as Vaseline.

Hair Loss (Alopecia)

There are many reasons why lupus may lead to hair loss. First of all, active

disease is associated with the plugging of hair follicles, which results in clumps of hair simply falling out after being combed or washed (called ‘‘lupus hair’’).

Patients with discoid lupus can experience mild, generalized hair loss, bald spots (alopecia areata), or even total baldness. Steroids may induce hair loss in the male pattern of baldness—in the temples and on top of the head. Also, infections, chemotherapy, emotional stress, and hormonal imbalances are associated

with hair loss. All told, about 30 percent of patients with SLE and DLE report

significant hair loss.

The treatment of alopecia depends on its cause. For example, discoid lesions

respond to local scalp injections with steroid preparations. If these areas form thick scars, hair may not regrow. Tapering off steroid use eliminates the ‘‘balding’’ pattern. Antimalarials and corticosteroids promote hair growth. Minoxidil (Rogaine) solution is a blood pressure preparation that promotes hair growth in balding men. It promotes hair growth in male and female lupus patients but

does not decrease hair loss.

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Butterfly (Malar) Rash

The term ‘‘lupus’’ was derived from the Latin word for ‘‘wolf’’ in an effort to describe one of the disease’s most recognizable features. About 35 percent of

patients with systemic or discoid lupus report a butterfly rash on their cheeks that suggests a wolf-like appearance. The rash reflects the angle at which the

ultraviolet radiation from the sun hits the skin. Rosacea, a sun-sensitive rash called polymorphous light eruption, and other disorders can be associated with

butterfly rashes, and many patients referred to me with a malar rash and a

suspected diagnosis of lupus turn out to have one of these conditions and not

lupus. Again, lesions in lupus generally do not itch and are identified as lupus under the microscope. One differentiating trick is that the nasolabial folds (where the outer parts of the nose and cheek meet the upper lip) are usually not affected in lupus. Occasionally, patients with a malar rash ignore their doctor’s advice and apply a great deal of fluorinated (e.g., Kenalog, Diprolene, Temovate) steroid salve for weeks or months. This results in thinning and wasting of the skin, which makes the condition look like a severe malar rash. The reason for this

are that capillaries (blood vessels) near the skin become more visible and mis-

lead the patient into believing that the lupus has worsened. Malar rashes from

lupus are treated with the judicious use of steroid ointments or gels, sun avoidance, and management of lupus activity in other parts of the body.

Changes in Pigmentation

Increased or decreased pigmentation of the skin is present in 10 percent of those with DLE or SLE. In other words, these patients may have areas of skin that

are darker or lighter than expected. As inflammation heals, patients may note

increases or decreases in pigment. Also, steroid deficiency (a decrease in the

steroids made by the adrenal gland) can increase pigmentation, as may anti-

malarial drugs.
Vitiligo
is an autoimmune skin condition associated with depig-mentation; it may be more common in those who have lupus. No systemic drug

is helpful for the pigmentation abnormalities of lupus, but some dermatologic

preparations applied to affected skin areas can improve the patient’s appearance.

Hives or Welts (Urticaria)

At some point in the course of their disease, 10 percent of patients with systemic lupus will develop hives. This is one of the few skin rashes of lupus that itch.

Most cases are related to coincidental allergic reactions, but an uncommon form of lupus may be associated with ‘‘lupus urticaria.’’ Most of these patients have deficiencies in certain blood complement components. For unclear reasons, lupus patients have an increased incidence of allergies in general (see Chapter 29).

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Where and How Can the Body Be Affected by Lupus?

Lupus urticaria is managed with antihistamines—H blockers such as hy-

1

droxyzine (Atarax, Zyrtec, Claritin, Benadryl) and H blockers such as cimeti-

2

dine (Tagamet)—antiserotonin drugs (Periactin), and steroids.

VASCULAR RASHES

Vasculitis
refers to inflammation of blood vessels, and such inflammation can lead to features detectable in the skin. Lupus usually involves the medium and

small-sized blood vessels. Small arteries and capillaries under the skin can be deprived of oxygen because of inflammation, abnormal vascular tone (which

controls whether a vessel dilates or constricts), or blood clots. A variety of

lesions associated with lupus stem from vascular problems.

Raynaud’s Phenomenon

One-third of patients with systemic lupus exhibit an unusual sign, referred to as
Raynaud’s phenomenon
, in which their fingers turn a patriotic red, white, and blue in response to stress, cold, or vibratory stimuli (e.g., jackhammers, pneu-matic drills). This usually reflects a malfunction of the autonomic nervous system’s ability to regulate the tone of the small blood vessels of the hand (whether they dilate or constrict). At times, Raynaud’s may be observed in the feet,

tongue, the tip of the nose, or on the outsides of the ears. There are many other causes of Raynaud’s, and it can be seen in most other rheumatic autoimmune

diseases. In fact, several surveys have shown that only about 9 percent of all

Raynaud’s is found among lupus patients. Raynaud’s can exist by itself (as

Raynaud’s disease
), but 30 to 40 percent of these patients have developed an autoimmune disease over a 10-year observation period, and some cases evolve

into lupus. Infrequently, Raynaud’s becomes so severe that the skin ulcerates

from lack of oxygen and gangrene may develop. Raynaud’s activity is usually

independent of lupus activity. (In other words, active SLE does not necessarily appear at the same time as active Raynaud’s, and vice versa.)

Raynaud’s is managed with preventive measures. These include wearing

gloves or mittens, avoiding cold environments, not smoking, and using hand

warmers. Medication such as beta-blockers, decongestants, and ergots given for

migraine headaches should be avoided or used sparingly. A variety of medica-

tions that increase the flow of blood to the hands may be prescribed, including nitroglycerine ointment, calcium channel blockers (diltiazem, or Cardizem, nifedipine (Procardia), nicardipine (Cardene), Amlodipine (Norvasc), sildenafil

(Viagra), bosentan (Tracleer), and other vasodilators such as reserpine or pra-

zosin (Minipress). Severe ulcerations of the fingers or gangrene can be treated with intravenous preparations such as prostaglandin. As a last-ditch effort to