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August 2016

Handout on Health:

Scleroderma

This publication is for people who have

scleroderma, as well as for their family

members, friends, and others who want to

find out more about the disease. This

publication describes the different forms of

scleroderma and provides information on

their symptoms, diagnosis, and treatment,

including what patients can do to help

manage their disease and the problems

associated with it. This publication also

highlights research efforts into the

understanding and treatment of scleroderma,

many of which are supported by the National

Institute of Arthritis and Musculoskeletal and

Skin Diseases (NIAMS) and other

components of the U.S. Department of

Health and Human Services’ National

Institutes of Health (NIH). If you have further

questions after reading this publication, you

may wish to discuss them with your doctor.

What Is Scleroderma?

What Are the Different Types of

Scleroderma?

What Causes Scleroderma?

Who Gets Scleroderma?

How Is Scleroderma Diagnosed?

How Is Scleroderma Treated?

Is Research Close to Finding a Cure?

More Questions? Count on More

Answers

For More Information

Key Words

Illustration

Types of Scleroderma

What Is Scleroderma?

We want to hear from you

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Derived from the Greek words “sklerosis,”

meaning hardness, and “derma,” meaning

skin, scleroderma literally means “hard skin.”

Although it is often referred to as if it were a

single disease, scleroderma is really a

symptom of a group of diseases that involve

the abnormal growth of connective tissue,

which supports the skin and internal organs.

In some forms of scleroderma, hard, tight

skin is the extent of this abnormal process. In

other forms, however, the problem goes

much deeper, affecting blood vessels and

internal organs, such as the heart, lungs, and

kidneys.

Scleroderma is called both a rheumatic

(roo-MA-tik) disease and a connective tissue

disease. The term rheumatic disease refers

to a group of conditions characterized by

inflammation or pain in the muscles, joints, or

fibrous tissue. A connective tissue disease is

one that affects tissues such as skin,

tendons, and cartilage.

Scleroderma is also believed to be an

autoimmune disease. In autoimmune

diseases, the body's immune system turns

against and damages its own tissues.

What Are the Different Types of

Scleroderma?

The group of diseases we call scleroderma

falls into two main classes: localized

scleroderma and systemic sclerosis.

(Localized diseases affect only certain parts

of the body; systemic diseases can affect the

whole body.) Both groups include subgroups

(see chart). Although there are different ways

these groups and subgroups may be broken

down or referred to (and your doctor may use

different terms from what you see here), the

following is a common way of classifying

these diseases:

Localized Scleroderma

Localized types of scleroderma are those

limited to the skin and related tissues and, in

some cases, the muscle below. Internal

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organs are not affected by localized

scleroderma, and localized scleroderma can

never progress to the systemic form of the

disease. Often, localized conditions improve

or go away on their own over time, but the

skin changes and damage that occur when

the disease is active can be permanent. For

some people, localized scleroderma is

serious and disabling.

There are two generally recognized types of

localized scleroderma:

Morphea: Morphea refers to local patches of

scleroderma. The first signs of the disease

are reddish patches of skin that thicken into

firm, oval-shaped areas. Patches appear

most often on the chest, stomach, and back.

Sometimes they appear on the face, arms,

and legs.

Morphea can be either localized or

generalized. Localized morphea limits itself to

one or several patches, ranging in size from

a half-inch to 12 inches in diameter. Some

people have both morphea and linear

scleroderma (described below). The disease

is referred to as generalized morphea when

the skin patches become very hard and dark

and spread over larger areas of the body.

Regardless of the type, morphea generally

fades out in 3 to 5 years; however, people

are often left with darkened skin patches and,

in rare cases, muscle weakness.

Linear scleroderma: As suggested by its

name, the disease is characterized by a

single line or band of thickened or abnormally

colored skin. Usually, the line runs down an

arm or leg, but in some people it runs down

the forehead.

Systemic Scleroderma (also known as

Systemic Sclerosis)

This is the term for the form of the disease

that not only includes the skin, but also

involves the tissues beneath, the blood

vessels, and the major organs. Systemic

sclerosis is typically broken down into limited

cutaneous scleroderma and diffuse

cutaneous scleroderma.

Limited cutaneous scleroderma: Limited

cutaneous scleroderma typically comes on

gradually and affects the skin only in certain

areas: the fingers, hands, face, lower arms,

and legs. People with limited disease often

have all or some of the symptoms that some

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doctors call CREST, which stands for the

following:

Calcinosis (KAL-sin-OH-sis): The

formation of calcium deposits in the

connective tissues, which can be

detected by x ray.

Raynaud’s (ray-NOHZ)

phenomenon: A condition in which

the small blood vessels of the hands

or feet contract in response to cold or

anxiety.

Esophageal (eh-SOFF-uh-GEE-ul)

dysfunction: Impaired function of the

esophagus (the tube connecting the

throat and the stomach) that occurs

when smooth muscles in the

esophagus lose normal movement.

Sclerodactyly (SKLER-oh-DAKtill-ee): Thick and tight skin on the

fingers, resulting from deposits of

excess collagen within skin layers.

Telangiectasia (tel-AN-jee-ekTAY-zee-uh): A condition caused by

the swelling of tiny blood vessels, in

which small red spots appear on the

hands and face.

Diffuse cutaneous scleroderma: This

condition typically comes on suddenly. Skin

thickening begins in the hands and spreads

quickly and over much of the body, affecting

the hands, face, upper arms, upper legs,

chest, and stomach in a symmetrical fashion

(for example, if one arm or one side of the

trunk is affected, the other is also affected).

Some people may have more area of their

skin affected than others. Internally, this

condition can damage key organs such as

the intestines, lungs, heart, and kidneys.

People with diffuse scleroderma face the

most serious long-term outlook if they

develop severe kidney, lung, digestive, or

heart problems. Fortunately, less than

one-third of patients with diffuse disease

develop these severe problems. Early

diagnosis and continual and careful

monitoring are important.

What Causes Scleroderma?

Although scientists don’t know exactly what

causes scleroderma, they are certain that

people cannot catch it from or transmit it to

others. Scientists suspect that scleroderma

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comes from several factors that may include:

Abnormal immune or inflammatory

activity: Like many other rheumatic

disorders, scleroderma is believed to be an

autoimmune disease. An autoimmune

disease is one in which the immune system,

for unknown reasons, turns against one’s

own body.

Genetic makeup: Although genes seem to

put certain people at risk for scleroderma and

play a role in its course, the disease is not

passed from parent to child like some genetic

diseases.

Environmental triggers: Researchers

suspect that exposure to some environmental

factors may trigger scleroderma.

Hormones: Women develop most types of

scleroderma more often than men. Scientists

suspect that hormonal differences between

women and men might play a part in the

disease.

Who Gets Scleroderma?

Although scleroderma is more common in

women, the disease also occurs in men and

children. It affects people of all races and

ethnic groups. However, there are some

patterns by disease type. For example:

Localized forms of scleroderma are

more common in people of European

descent than in African Americans.

Morphea usually appears between

the ages of 20 and 40, and linear

scleroderma usually occurs in

children or teenagers.

Systemic scleroderma, whether

limited or diffuse, typically occurs in

people from 30 to 50 years old. It

affects more women of African

American than European descent.

Because scleroderma can be hard to

diagnose and it overlaps with or resembles

other diseases, scientists can only estimate

how many cases there actually are. It is

estimated that 49,000 adults in the United

States have systemic sclerosis.1

1 Helmick CG, Felson DT, Lawrence RC,

Gabriel S, Hirsch R, Kwoh CK, Liang MH,

Kremers M, Mayes MD, Merkel PA, Pillemer

SR, Reveille JD, Stone JH; National Arthritis

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Data Workgroup. Estimates of the prevalence

of arthritis and other rheumatic conditions in

the United States. Part I. Arthritis Rheum.

2008 Jan;58(1):15-25.

For some people, scleroderma (particularly

the localized forms) is fairly mild and resolves

with time. But for others, living with the

disease and its effects day to day has a

significant impact on their quality of life.

How Is Scleroderma Diagnosed?

Depending on your particular symptoms, a

diagnosis of scleroderma may be made by:

A general internist.

A dermatologist, who specializes in

treating diseases of the skin, hair, and

nails.

An orthopaedist, who treats bone

and joint disorders.

A pulmonologist, who is trained to

treat lung problems.

A rheumatologist, who specializes in

treating musculoskeletal disorders

and rheumatic diseases.

A diagnosis of scleroderma is based largely

on the medical history and findings from the

physical exam, questions about what has

happened to you over time, and about any

symptoms you may be experiencing. Once

your doctor has taken a thorough medical

history, he or she will perform a physical

exam, which can help the doctor diagnose a

certain form of scleroderma.

Finally, your doctor may order lab tests to

help confirm a suspected diagnosis. The

presence of certain antibodies is common in

people with scleroderma, but having these

antibodies does not confirm the presence of

the disease.

In some cases, your doctor may order a skin

biopsy (the surgical removal of a small

sample of skin for microscopic examination)

to aid in or help confirm a diagnosis.

However, skin biopsies also have their

limitations: biopsy results cannot distinguish

between localized and systemic disease, for

example.

Diagnosing scleroderma is easiest when a

person has typical symptoms and rapid skin

thickening. In other cases, a diagnosis may

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take months, or even years, as the disease

unfolds and reveals itself and as the doctor is

able to rule out some other potential causes

of the symptoms.

How Is Scleroderma Treated?

Because scleroderma can affect many

different organs and organ systems, you may

have several different doctors involved in

your care. Typically, care will be managed by

a rheumatologist (a doctor specializing in

treatment of musculoskeletal disorders and

rheumatic diseases). Your rheumatologist

may refer you to other specialists, depending

on the specific problems you are having. For

example, you may see a dermatologist for

the treatment of skin symptoms, a

nephrologist for kidney complications, a

cardiologist for heart complications, a

gastroenterologist for problems of the

digestive tract, and a pulmonary specialist for

lung involvement.

In addition to doctors, professionals such as

nurse practitioners, physician assistants,

physical or occupational therapists,

psychologists, and social workers may play a

role in your care. Dentists, orthodontists, and

even speech therapists can treat oral

complications that arise from thickening of

tissues in and around the mouth and on the

face.

Currently, there is no treatment that controls

or stops the underlying problem—the

overproduction of collagen—in all forms of

scleroderma. Thus, treatment and

management focus on relieving symptoms

and limiting damage. Your treatment will

depend on the particular problems you are

having. Some treatments will be prescribed

or given by your doctor. Others are things

you can do on your own.

Stiff, painful joints: In diffuse systemic

sclerosis, hand joints can stiffen because of

hardened skin around the joints or

inflammation within them. Other joints can

also become stiff and swollen.

Stretching exercises under the

direction of a physical or occupational

therapist are extremely important to

prevent loss of joint motion.

Exercise regularly. Ask your doctor or

physical therapist about an exercise

plan that will help you increase and

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maintain range of motion in affected

joints.

Use medication as recommended by

your doctor to help relieve joint or

muscle pain.2 If pain is severe, speak

to a rheumatologist about the

possibility of prescription-strength

drugs to ease pain and inflammation.

Learn to do things in a new way. A

physical or occupational therapist can

help you learn to perform daily tasks,

such as lifting and carrying objects or

opening doors, in ways that will put

less stress on tender joints.

2 All medicines can have side effects. Some

medicines and side effects are mentioned in

this publication. Some side effects may be

more severe than others. You should review

the package insert that comes with your

medicine and ask your health care provider

or pharmacist if you have any questions

about the possible side effects.

Skin problems: When too much collagen

builds up in the skin, it crowds out sweat and

oil glands, causing the skin to become dry

and stiff. If your skin is affected, try the

following:

Apply oil-based creams and lotions

frequently, and always right after

bathing.

Apply sunscreen before you venture

outdoors to protect against further

damage from the sun’s rays.

Use humidifiers to moisten the air in

your home in colder winter climates.

Clean humidifiers often to stop

bacteria from growing in the water.

Avoid very hot baths and showers, as

hot water dries the skin.

Avoid harsh soaps, household

cleaners, and caustic chemicals, if at

all possible. Otherwise, be sure to

wear rubber gloves when you use

such products.

Exercise regularly. Exercise,

especially swimming, stimulates

blood circulation to affected areas.

Dry mouth and dental problems: Dental

problems are common in people with

scleroderma for a number of reasons:

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Tightening facial skin can make the

mouth opening smaller and narrower,

which makes it hard to care for teeth.

Dry mouth caused by salivary gland

damage speeds up tooth decay.

Damage to connective tissues in the

mouth can lead to loose teeth.

You can avoid tooth and gum problems in

several ways:

Brush and floss your teeth regularly. If

hand pain and stiffness make this

difficult, consult your doctor or an

occupational therapist about specially

made toothbrush handles and

devices to make flossing easier.

Have regular dental checkups.

Contact your dentist immediately if

you experience mouth sores, mouth

pain, or loose teeth.

If decay is a problem, ask your dentist

about fluoride rinses or prescription

toothpastes that remineralize and

harden tooth enamel.

Consult a physical therapist about

facial exercises to help keep your

mouth and face more flexible.

Keep your mouth moist by drinking

plenty of water, sucking ice chips,

using sugarless gum and hard candy,

and avoiding mouthwashes with

alcohol. If dry mouth still bothers you,

ask your doctor about a saliva

substitute or medications that can

stimulate the flow of saliva.

Gastrointestinal (GI) problems: Systemic

sclerosis can affect any part of the digestive

system. As a result, you may experience

problems such as heartburn, difficulty

swallowing, early satiety (the feeling of being

full after you’ve barely started eating), or

intestinal complaints such as diarrhea,

constipation, and gas. In cases where the

intestines are damaged, your body may have

difficulty absorbing nutrients from food.

Although GI problems are diverse, here are

some things that might help at least some of

the problems you have:

Eat small, frequent meals.

To keep stomach contents from

backing up into the esophagus, stand

or sit for at least an hour (preferably 2

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or 3 hours) after eating.

When it is time to sleep, keep the

head of your bed raised using blocks.

Avoid late-night meals, spicy or fatty

foods, alcohol, and caffeine, which

can aggravate GI distress.

Eat moist, soft foods, and chew them

well. If you have difficulty swallowing

or if your body doesn’t absorb

nutrients properly, your doctor may

prescribe a special diet.

Ask your doctor about medications for

problems such as diarrhea,

constipation, and heartburn. Some

drugs called proton pump inhibitors

are highly effective against heartburn.

Oral antibiotics may stop bacterial

overgrowth in the bowel, which can

be a cause of diarrhea in some

people with systemic sclerosis.

Lung damage: Virtually all people with

systemic sclerosis have some loss of lung

function. Some develop severe lung disease,

which comes in two forms: pulmonary fibrosis

(hardening or scarring of lung tissue because

of excess collagen) and pulmonary

hypertension (high blood pressure in the

artery that carries blood from the heart to the

lungs). Treatment for the two conditions is

different:

Pulmonary fibrosis may be treated

with drugs that suppress the immune

system, along with low doses of

corticosteroids.

Pulmonary hypertension may be

treated with drugs that dilate the

blood vessels or with newer

medications that are prescribed

specifically for treating pulmonary

hypertension.

Regardless of your particular lung problem or

its medical treatment, your role in the

treatment process is essentially the same. To

minimize lung complications, work closely

with your medical team. Do the following:

Watch for signs of lung disease,

including fatigue, shortness of breath

or difficulty breathing, and swollen

feet. Report these symptoms to your

doctor.

Have your lungs closely checked,

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using standard lung-function tests,

during the early stages of skin

thickening. These tests, which can

find problems at the earliest and most

treatable stages, are needed because

lung damage can occur even before

you notice any symptoms.

Get regular flu and pneumonia

vaccines as recommended by your

doctor. Contracting either illness

could be dangerous for a person with

lung disease.

Heart problems: Common among people

with scleroderma, heart problems include

scarring and weakening of the heart

(cardiomyopathy), inflamed heart muscle

(myocarditis), and abnormal heartbeat

(arrhythmia). All of these problems can be

treated. Treatment ranges from drugs to

surgery and varies depending on the nature

of the condition.

Kidney problems: Renal crisis is an

uncommon but serious complication in

patients with systemic sclerosis. Renal crisis

results in severe uncontrolled high blood

pressure, which can quickly lead to kidney

failure. It’s very important that you take

measures to identify and treat the

hypertension as soon as it occurs. These are

things you can do:

Check your blood pressure regularly.

You should also check it if you have

any new or different symptoms such

as a headache or shortness of breath.

If your blood pressure is higher than

usual, call your doctor right away.

If you have kidney problems, take

your prescribed medications faithfully.

In the past two decades, drugs known

as ACE (angiotensin-converting

enzyme) inhibitors have made

scleroderma-related kidney failure a

less threatening problem than it used

to be. But for these drugs to work,

you must take them as soon as the

hypertension is present.

Cosmetic problems: Even if scleroderma

doesn’t cause any lasting physical disability,

its effects on the skin’s appearance

—particularly on the face—can take their toll

on your self-esteem. Fortunately, there are

procedures to correct some of the cosmetic

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problems scleroderma causes:

The appearance of telangiectasias

—small red spots on the hands and

face caused by swelling of tiny blood

vessels beneath the skin—may be

reduced or even eliminated with the

use of guided lasers.

Facial changes of localized

scleroderma—such as the en coup de

sabre that may run down the

forehead in people with linear

scleroderma—may be corrected

through cosmetic surgery. (However,

such surgery is not appropriate for

areas of the skin where the disease is

active.)

Is Research Close to Finding a

Cure?

No one can say for sure when—or if—a cure

will be found. But research is providing the

next best thing: better ways to treat

symptoms, prevent organ damage, and

improve the quality of life for people with

scleroderma. Multidisciplinary research has

also provided new clues for understanding

the disease, which is an important step

toward prevention and cure.

Studies of the immune system, genetics, cell

biology, and molecular biology have helped

reveal the causes of scleroderma, improve

existing treatment, and create entirely new

treatment approaches.

Some advances in the understanding or

treatment of scleroderma include the

following:

Building on research that identified a

gene associated with scleroderma in

Oklahoma Choctaw Native

Americans, scientists are using new

technology to look for other genes

associated with the disease’s

development and severity.

The drug cyclophosphamide has

been found effective in treating lung

fibrosis. One study suggested that

treating lung problems early with this

immunosuppressive drug may help

prevent further damage and increase

chances of survival. Clinical trials

assessing the effectiveness of other

medications for lung fibrosis are

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ongoing.

ACE inhibitors are used increasingly

for scleroderma-related kidney

problems. ACE inhibitors have greatly

reduced the risk of kidney failure in

people with scleroderma.

Several drugs are now available to

treat pulmonary hypertension.

Previously, pulmonary hypertension

was associated with a poor outcome,

but medications have increased the

quality of life and life expectancy for

people with this dangerous form of

lung damage.

Other studies are examining the following:

The immune system: Researchers

are looking at the complex immune

system trying to determine why the

body creates antibodies against its

own tissues. Identifying where and

when the antibodies are created and

how they function could lead

researchers to find targets for new

therapies. Researchers are already

trying new therapies, including those

that suppress specific parts of the

immune system.

Genes: Studies continue to find the

genes that predispose a person to

getting scleroderma. Those genes

may be targets for future therapies.

Researchers are also trying to find

genes (and other indicators) that

could tell doctors who will develop

what type of scleroderma. This could

someday help doctors identify who

might benefit from specific therapies.

Additionally, since genes are not the

only factor involved in scleroderma,

researchers are looking for

environmental factors that might

trigger the disease in those who are

genetically predisposed.

Drug therapies: Through basic

studies of the molecular and genetic

basis of scleroderma, scientists have

indentified a large number of potential

therapeutic targets. Other

researchers are testing existing drugs

to see if they are succesful in

delaying cardiovascular changes.

Animal models: Experiments using

mice have proved valuable in past

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research on scleroderma. Further

research using existing strains of

mice continues.

The fibrosis process: Researchers

are looking into the many factors that

lead to fibrosis to see if they can

block its development.

Cytokines: Studies have shown that

certain chemicals called cytokines,

made from cells in the body,

contribute to inflammation.

Researchers are trying to determine

how cytokines contribute to the

disease process.

More information on research is available

from the following websites:

NIH Clinical Research Trials and

You was designed to help people

learn more about clinical trials, why

they matter, and how to participate.

Visitors to the website will find

information about the basics of

participating in a clinical trial,

first-hand stories from clinical trial

volunteers, explanations from

researchers, and links on how to

search for a trial or enroll in a

research-matching program.

ClinicalTrials.gov offers up-to-date

information for locating federally and

privately supported clinical trials for a

wide range of diseases and

conditions.

NIH RePORTER is an electronic tool

that allows users to search a

repository of both intramural and

extramural NIH-funded research

projects from the past 25 years and

access publications (since 1985) and

patents resulting from NIH funding.

PubMed is a free service of the U.S.

National Library of Medicine that lets

you search millions of journal citations

and abstracts in the fields of

medicine, nursing, dentistry,

veterinary medicine, the health care

system, and preclinical sciences.

More Questions? Count on More

Answers

Scleroderma poses a series of challenges for

both patients and their health care teams.

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The good news is that scientists, doctors,

and other health care professionals continue

to find new ways to make earlier diagnoses

and manage disease better. In addition,

active patient support groups share with, care

for, and educate each other. The impact of all

of this activity is that people with scleroderma

do much better and remain active far longer

than they did 20 or 30 years ago. As for

tomorrow, patients and the medical

community will continue to push for longer,

healthier, and more active lives for people

with the diseases collectively known as

scleroderma.

For More Information

National Institute of Arthritis and

Musculoskeletal and Skin Diseases

(NIAMS)

Information Clearinghouse

National Institutes of Health

1 AMS Circle

Bethesda, MD 20892-3675

Phone: 301-495-4484

Toll free: 877-22-NIAMS (877-226-4267)

TTY: 301-565-2966

Fax: 301-718-6366

Email: NIAMSinfo@mail.nih.gov

Website: https://www.niams.nih.gov

If you need more information about available

resources in your language or another

language, please visit our website or contact

the NIAMS Information Clearinghouse at

NIAMSinfo@mail.nih.gov.

Other Resources

American Academy of Dermatology

Website: http://www.aad.org

American College of Rheumatology

Website: http://www.rheumatology.org

Scleroderma Foundation

Website: http://www.scleroderma.org (con

información en español)

Scleroderma Research Foundation

Website: http://www.srfcure.org

Arthritis Foundation

Website: http://www.arthritis.org

Key Words

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Antibodies. Special proteins produced by

the body’s immune system. They recognize

and help fight infectious agents, such as

bacteria and other foreign substances that

invade the body. The presence of certain

antibodies in the blood can help to diagnose

some diseases, including some forms of

scleroderma.

Autoimmune disease. A disease in which

the body’s immune system turns against and

damages its own tissues.

Calcinosis. The formation of calcium

deposits in the connective tissues, which can

be detected by x ray. These deposits are

typically found on the fingers, hands, face,

and trunk and on the skin above elbows and

knees. When the deposits break through the

skin, painful ulcers can result.

Collagen. A fabric-like material of fibrous

threads that is a key component of the body’s

connective tissues. In scleroderma, either too

much collagen is produced or it is produced

in the wrong places, causing stiff and

inflamed skin, blood vessels, and internal

organs.

Connective tissue. Tissues such as skin,

tendons, and cartilage that support and hold

body parts together. The chief component of

connective tissue is collagen.

CREST syndrome. An acronym for a

collection of symptoms that occur to some

degree in all people with systemic sclerosis.

The symptoms are calcinosis, Raynaud’s

phenomenon, esophageal dysfunction,

sclerodactyly, and telangiectasia. Because of

the predominance of CREST symptoms in

people with limited systemic sclerosis, some

people use the term CREST syndrome when

referring to that form of the disease.

Fibrosis. A condition marked by increased

fibrous tissue that develops between the cells

of various organs or tissues. It is a common

feature of scleroderma and some other

diseases. Fibrosis causes hardening or

stiffening of tissues in the skin, joints, and

internal organs.

Pulmonary fibrosis. Hardening or scarring

of lung tissue because of excess collagen.

Pulmonary fibrosis occurs in a small

percentage of people with systemic sclerosis.

Pulmonary hypertension. Abnormally high

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blood pressure in the arteries supplying the

lungs that may be caused by a number of

factors, including damage from fibrosis.

Raynaud’s phenomenon. A condition in

which the small blood vessels of the hands or

feet contract in response to cold or anxiety.

As the vessels contract, the hands or feet

turn white and cold, then blue. As blood flow

returns, they become red. Fingertip tissues

may suffer damage, leading to ulcers, scars,

or gangrene.

Rheumatic. An adjective used to describe a

group of conditions characterized by

inflammation or pain in the muscles, joints,

and fibrous tissue. Rheumatic diseases or

disorders can be related to autoimmunity or

other causes.

Sclerodactyly. Thick and tight skin on the

fingers, resulting from deposits of excess

collagen within skin layers. The condition

makes it harder to bend or straighten the

fingers. The skin may also appear shiny and

darkened, with hair loss.

Telangiectasia. A condition caused by the

swelling of tiny blood vessels, in which small

red spots appear on the hands and face.

Although not painful, these red spots can

create cosmetic problems.

Acknowledgments

The NIAMS gratefully acknowledges the

assistance of the following individuals in the

preparation and review of previous versions

of this publication: Reva Lawrence, M.P.H.,

Barbara Mittleman, M.D., Alan Moshell, M.D.,

and Susana Serrate-Sztein, M.D.,

NIAMS/NIH; Stanley Pillemer, M.D., National

Institute of Dental and Craniofacial Research,

NIH; Philip Clements, M.D., University of

California at Los Angeles, CA; Vincent

Falanga, M.D., Boston University, Boston,

MA; E. Carwile LeRoy, M.D., Medical

University of South Carolina, Charleston, SC;

Morris Reichlin, M.D., Oklahoma Medical

Research Foundation, Oklahoma City, OK;

Larry Solomon, M.D., Lutheran General

Children’s Hospital, Park Ridge, IL; Virginia

Steen, M.D., Georgetown University Medical

Center, Washington, DC; Barbara White,

M.D., University of Maryland at Baltimore,

Catonsville, MD; and the Scleroderma

Foundation, Danvers, MA. Special thanks

also go to the patients who reviewed this

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publication and provided valuable assistance.

The mission of the National Institute of

Arthritis and Musculoskeletal and Skin

Diseases (NIAMS), a part of the U.S.

Department of Health and Human Services’

National Institutes of Health (NIH), is to

support research into the causes, treatment,

and prevention of arthritis and

musculoskeletal and skin diseases; the

training of basic and clinical scientists to

carry out this research; and the dissemination

of information on research progress in these

diseases. The NIAMS Information

Clearinghouse is a public service sponsored

by the Institute that provides health

information and information sources.

Additional information can be found on the

NIAMS website at http://www.niams.nih.gov.

For Your Information

This publication contains information about

medications used to treat the health condition

discussed here. When this publication was

developed, we included the most up-to-date

(accurate) information available.

Occasionally, new information on medication

is released.

For updates and for any questions about any

medications you are taking, please contact

U.S. Food and Drug Administration

Toll free: 888-INFO-FDA

(888-463-6332)

Website: http://www.fda.gov

For additional information on specific

medications, visit Drugs@FDA at

http://www.accessdata.fda.gov/scripts

/cder/daf/. Drugs@FDA is a searchable

catalog of FDA-approved drug products.

For updates and questions about statistics,

please contact

Centers for Disease Control and

Prevention, National Center for

Health Statistics

Toll free: 800-232-4636

Website: http://www.cdc.gov/nchs

This publication is not copyrighted. Readers

are encouraged to duplicate and distribute as

many copies as needed.

Additional copies of this publication are

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available from:

National Institute of Arthritis and

Musculoskeletal and Skin Diseases

(NIAMS)

Information Clearinghouse

National Institutes of Health

1 AMS Circle

Bethesda, MD 20892-3675

Phone: 301-495-4484

Toll free: 877-22-NIAMS

(877-226-4267)

TTY: 301-565-2966

Fax: 301-718-6366

Email: NIAMSinfo@mail.nih.gov

Website: https://www.niams.nih.gov

NIH Publication No. 16–4271

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available in print. Would you like

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