Make a donation to Wikipedia and give the gift of knowledge!

Scleroderma
From Wikipedia, the free encyclopedia

Jump to: navigation, search

This article is about the disease. For the mushroom, see Scleroderma (genus).
Scleroderma
Classification and external
resources

ICD-10 L94.0-L94.1,
M34.

ICD-9 701.0 710.1

OMIM 181750

DiseasesDB 12845

MedlinePlus 000429

eMedicine med/2076
med/3132
derm/677
ped/2197
Scleroderma is a chronic autoimmune disease characterized by a hardening[1] or
sclerosis[2] in the skin or other organs. The localized type of the disease, known as
"morphea",[3] while disabling, tends not to be fatal. The systemic type or systemic
sclerosis, the generalized type of the disease, can be fatal as a result of heart, kidney, lung
or intestinal damage.[4]

Contents
[hide]

• 1 Signs and symptoms

o 1.1 Skin symptoms
o 1.2 Other organs
• 2 Diagnosis
• 3 Types
o 3.1 Diffuse scleroderma
o 3.2 Limited scleroderma/CREST syndrome
o 3.3 Morphea/linear scleroderma
• 4 Causes
• 5 Pathophysiology
• 6 Therapy
o 6.1 Topical/symptomatic
o 6.2 Kidney disease
o 6.3 Lung disease and pulmonary hypertension
o 6.4 Experimental treatments
• 7 Epidemiology
• 8 Advocacy
• 9 References

• 10 External links

[edit] Signs and symptoms

[edit] Skin symptoms

In the skin, scleroderma causes hardening and scarring. The skin may appear tight,
reddish or scaly. Blood vessels may also be more visible. Where large areas are affected,
fat and muscle wastage may weaken limbs and affect appearance. There is much
variation in severity between people with the condition, with some having scleroderma of
only a limited area of the skin (such as the fingers) and little involvement of the
underlying tissue.[citation needed]

[edit] Other organs

Diffuse scleroderma can cause musculoskeletal, pulmonary, gastrointestinal, renal and
other complications.[4]Patients with larger amounts of cutaneous involvement are more
likely to have involvement of the internal tissues and organs. Most patients (over 80%)
have vascular symptoms and Raynaud's phenomenon, which leads to attacks of
discoloration of the hands and feet in response to cold. Raynaud's normally affects the
fingers and toes. Systemic scleroderma and Raynaud's can cause painful ulcers on the
fingers or toes which are known as digital ulcers. Calcinosis (deposition of calcium in
lumps under the skin) is also common in systemic scleroderma, and is often seen near the
elbows, knees or other joints.

Musculoskeletal

The first joint symptoms that patients with scleroderma have are typically non specific
joint pains, which can lead to arthritis, or cause discomfort in tendons or muscles.[4] Joint
mobility, especially of the small joints of the hand, may be restricted by calcinosis or skin
thickening.[5] Patients may develop muscle weakness, or myopathy, either from the
disease, or its treatments.[6]

Lungs

Some impairment in lung function is almost universally seen in patients with diffuse
scleroderma on pulmonary function testing;[7] however, it does not necessarily cause
symptoms, such as shortness of breath. Some patients can develop pulmonary
hypertension, or elevation in the pressures of the pulmonary arteries. This can be
progressive, and lead to right sided heart failure. The earliest manifestation of this may be
a decreased diffusion capacity on pulmonary function testing.

Other pulmonary complications in more advanced disease include aspiration pneumonia,

pulmonary hemorrhage and pneumothorax.[4]

Digestive tract

Endoscopic image of peptic stricture, or narrowing of the esophagus near the junction
with the stomach due to chronic gastroesophageal reflux. This is the most common cause
of dysphagia, or difficulty swallowing, in scleroderma.

Diffuse scleroderma can affect any part of the gastrointestinal tract.[8] The most common
manifestation in the esophagus is reflux esophagitis, which may be complicated by peptic
stricturing, or benign narrowing of the esophagus.[9] This is best initially treated with
proton pump inhibitors for acid suppression,[10] but may require bougie dilatation in the
case of stricture.[8]

Scleroderma can decrease motility anywhere in the gastrointestinal tract.[8] The most
common source of decreased motility involvement is the esophagus and the lower
esophageal sphincter, leading to dysphagia and chest pain. As Scleroderma progresses,
esophageal involvement from abnormalities in decreased motility may worsen due to
progressive fibrosis (scarring). If this is left untreated, acid from the stomach can back up
into the esophagus causing esophagitis, and GERD. Further scarring from acid damage to
the lower esophagus many times leads to the development of fibrotic narrowing, also
known as strictures which can be treated by dilitation, and Barrett's esophagus. The small
intestine can also become involved, leading to bacterial overgrowth and malabsorption,
of bile salts, fats, carbohydrates, proteins, and vitamins. The colon can be involved, and
can cause pseudo-obstruction or ischemic colitis.[4]

Rarer complications include pneumatosis cystoides intestinalis, or gas pockets in the

bowel wall, wide mouthed diverticula in the colon and esophagus, and liver fibrosis.
Patients with severe gastrointestinal involvement can become profoundly malnourished.[9]

Scleroderma may also be associated with gastric antral vascular ectasia (GAVE), also
known as watermelon stomach. This is a condition where atypical blood vessels
proliferate usually in a radially symmetric pattern around the pylorus of the stomach.
GAVE can be a cause of upper gastrointestinal bleeding or iron deficiency anemia in
patients with scleroderma.[9]

Kidneys

Renal involvement, in scleroderma, is considered a poor prognostic factor and frequently

a cause of death.[11]

The most important clinical complication of scleroderma involving the kidney is

scleroderma renal crisis. Symptoms of scleroderma renal crisis are malignant
hypertension (high blood pressure with evidence of acute organ damage), hyperreninemia
(high renin levels), azotemia (kidney failure with accumulation of waste products in the
blood) and microangiopathic hemolytic anemia (destruction of red blood cells).[12] Apart
from the high blood pressure, hematuria (blood in the urine) and proteinuria (protein loss
in the urine) may be indicative.[13]

In the past scleroderma renal crisis was almost uniformily fatal.[14] While outcomes have
improved significantly with the use of ACE inhibitors[15][16] the prognosis is often
guarded, as a significant number of patients are refractory to treatment and develop renal
failure. Approximately 5-10% of all scleroderma patients develop renal crisis at some
point in the course of their disease.[17] Patients that have rapid skin involvement have the
highest risk of renal complications.[17] It is most common in diffuse cutaneous
scleroderma, and is often associated with antibodies against RNA polymerase (in 59% of
cases). Many proceed to dialysis, although this can be stopped within three years in about
a third of cases. Higher age and (paradoxically) a lower blood pressure at presentation
make it more likely that dialysis is needed.[18]

Treatments for scleroderma renal crisis include ACE inhibitors, which are also used for
prophylaxis,[17][16] and renal transplantation. Transplanted kidneys are known to be
affected by scleroderma and patients with early onset renal disease (within one year of
the scleroderma diagnosis) are thought to have the highest risk for recurrence.[19]

[edit] Diagnosis
Diagnosis is by clinical suspicion, presence of autoantibodies (specifically anti-
centromere and anti-scl70/anti-topoisomerase antibodies) and occasionally by biopsy. Of
the antibodies, 90% have a detectable anti-nuclear antibody. Anti-centromere antibody is
more common in the limited form (80-90%) than in the systemic form (10%), and anti-
scl70 is more common in the diffuse form (30-40%) and in African-American patients
(who are more susceptible to the systemic form).[20]

In 1980 the American College of Rheumatology agreed upon diagnostic criteria for
scleroderma.[21]

[edit] Types
There are three major forms of scleroderma: diffuse, limited (CREST syndrome) and
morphea/linear. Diffuse and limited scleroderma are both a systemic disease, whereas the
linear/morphea form is localized to the skin. (Some physicians consider CREST and
limited scleroderma one and the same, others treat them as two separate forms of
scleroderma.) There is also a subset of the systemic form known as "systemic
scleroderma sine scleroderma", meaning the usual skin involvement is not present.

[edit] Diffuse scleroderma

Diffuse scleroderma (progressive systemic sclerosis) is the most severe form - it has a
rapid onset, involves more widespread skin hardening, will generally cause much internal
organ damage (specifically the lungs and gastrointestinal tract), and is generally more life
threatening.

[edit] Limited scleroderma/CREST syndrome

The limited form is much milder: it has a slow onset and progression, skin hardening is
usually confined to the hands and face, internal organ involvement is less severe, and a
much better prognosis is expected.

Sclerotic piece-meal necrosis of the tip of the thumb in a patient with scleroderma.
In typical cases of limited scleroderma, Raynaud's phenomenon may precede scleroderma
by several years. Raynaud's phenomenon is due to vasoconstriction of the small arteries
of exposed peripheries - particularly the hands and feet - in the cold. It is classically
characterised by a triphasic colour change - first white, then blue and finally red on
rewarming. The scleroderma may be limited to the fingers - known as sclerodactyly.

The limited form is often referred to as CREST syndrome.[22] "CREST" is an acronym for
the five main features:

• Calcinosis
• Raynaud's syndrome
• Esophageal dysmotility
• Sclerodactyly
• Telangiectasia

CREST is a limited form associated with antibodies against centromeres and usually
spares the lungs and kidneys.

[edit] Morphea/linear scleroderma

Morphea/linear scleroderma involves isolated patches of hardened skin - there generally

is no internal organ involvement.[23]

[edit] Causes
There is no clear obvious cause for scleroderma and systemic sclerosis. Genetic
predisposition appears to be limited: genetic concordance is small; still, there often is a
familial predisposition for autoimmune disease. Polymorphisms in COL1A2 and TGF-β1
may influence severity and development of the disease. There is limited evidence
implicating cytomegalovirus (CMV) as the original epitope of the immune reaction, and
organic solvents and other chemical agents have been linked with scleroderma.[20]

One of the suspected mechanisms behind the autoimmune phenomenon is the existence
of microchimerism, i.e. fetal cells circulating in maternal blood, triggering an immune
reaction to what is perceived as "foreign" material.[24][20]

A distinct form of scleroderma and systemic sclerosis may develop in patients with
chronic renal failure. This entity, nephrogenic fibrosing dermopathy or nephrogenic
systemic fibrosis,[25] has been linked to the exposure to gadolinium-containing
radiocontrast.[26]

Bleomycin[27] (a chemotherapeutic agent) and possibly taxane chemotherapy[28] may cause

scleroderma, and occupational exposure to solvents has been linked with an increased
risk of systemic sclerosis.[29]
[edit] Pathophysiology
The overproduction of collagen is thought to result from an autoimmune dysfunction, in
which the immune system would start to attack the kinetochore of the chromosomes. This
would lead to genetic malfunction of nearby genes. T cells accumulate in the skin; these
are thought to secrete cytokines and other proteins that stimulate collagen deposition.
Stimulation of the fibroblast, in particular, seems to be crucial to the disease process, and
studies have converged on the potential factors that produce this effect.[20]

A significant player in the process is transforming growth factor (TGFβ). This protein
appears to be overproduced, and the fibroblast (possibly in response to other stimuli) also
overexpresses the receptor for this mediator. An intracellular pathway (consisting of
SMAD2/SMAD3, SMAD4 and the inhibitor SMAD7) is responsible for the secondary
messenger system that induces transcription of the proteins and enzymes responsible for
collagen deposition. Sp1 is a transcription factor most closely studied in this context.
Apart from TGFβ, connective tissue growth factor (CTGF) has a possible role.[20] Indeed,
a common CTGF gene polymorphism is present at an increased rate in systemic
sclerosis.[30]

Damage to endothelium is an early abnormality in the development of scleroderma, and

this too seems to be due to collagen accumulation by fibroblasts, although direct
alterations by cytokines, platelet adhesion and a type II hypersensitivity reaction have
similarly been implicated. Increased endothelin and decreased vasodilation has been
documented.[20]

Jimenez & Derk[20] describe three theories about the development of scleroderma:

• The abnormalities are primarily due to a physical agent, and all other changes are
secondary or reactive to this direct insult.
• The initial event is fetomaternal cell transfer causing microchimerism, with a
second summative cause (e.g. environmental) leading to the actual development
of the disease.
• Physical causes lead to phenotypic alterations in susceptible cells (e.g. due to
genetic makeup), which then effectuate DNA changes which alter the cell's
behavior.

[edit] Therapy
There is no cure for every patient with scleroderma, though there is treatment for some of
the symptoms, including drugs that soften the skin and reduce inflammation. Some
patients may benefit from exposure to heat.[31]

[edit] Topical/symptomatic
Topical treatment for the skin changes of scleroderma do not alter the disease course, but
may improve pain and ulceration. A range of NSAIDs (nonsteroidal anti-inflammatory
drugs) can be used to ease painful symptoms, such as naproxen.[citation needed] There is
limited benefit from steroids such as prednisone.[citation needed] Episodes of Raynaud's
phenomenon sometimes respond to nifedipine or other calcium channel blockers; severe
digital ulceration may respond to prostacyclin analogue iloprost, and the dual endothelin-
receptor antagonist bosentan may be beneficial for Raynaud's phenomenon.[32] The skin
tightness may be treated systemically with methotrexate and cyclosporin.[32]

[edit] Kidney disease

Scleroderma renal crisis, the occurrence of acute renal failure and malignant hypertension
(very high blood pressure with evidence of organ damage) in people with scleroderma, is
effectively treated with drugs from the class of the ACE inhibitors. The benefit of ACE
inhibitors extends even to those who have to commence dialysis to treat their kidney
disease, and may give sufficient benefit to allow the discontinuation of renal replacement
therapy.[32]

[edit] Lung disease and pulmonary hypertension

Active alveolitis is often treated with pulses of cyclophosphamide, often together with a
small dose of steroids. The benefit of this intervention is modest.[33][34]

Pulmonary hypertension may be treated with epoprostenol, bosentan and possibly

aerolized iloprost.[32]

[edit] Experimental treatments

Given the difficulty in treating scleroderma, treatments with a smaller evidence base are
often tried to control the disease. These include antithymocyte globulin and
mycophenolate mofetil; some reports have reported improvements in the skin symptoms
as well as delaying the progress of systemic disease, but neither of them have been
subjected to large clinical trials.[32]

While still experimental (given its high rate of complications), hematopoietic stem cell
transplantation is being studied in patients with severe systemic sclerosis; improvement
in life expectancy and severity of skin changes has been noted.[35]

[edit] Epidemiology
The examples and perspective in this article or section may not represent a worldwide
view of the subject.
Please improve this article or discuss the issue on the talk page. (May 2008)
Scleroderma affects approximately 300,000 people in the United States. It is four times as
common in women than in men. Incidence rates are estimated at 2-20 per million per year
in the United States.[citation needed]

Juvenile scleroderma affects approximately 7000 children in the United States. The most
common form of juvenile scleroderma is localized scleroderma, morphea and/or linear.

[edit] Advocacy
The Juvenile Scleroderma Network is an organization dedicated to provide emotional
support and educational information to parents and their children living with juvenile
scleroderma, to support pediatric research to identify the cause of and the cure for
juvenile sscleroderma, and to enhance public awareness.[36]

In the US, the Scleroderma Research Foundation is dedicated to raise awareness of the
disease and assist those who are affected.[37] The Scleroderma Research Foundation
sponsors research into the condition.[38] Comedian and television presenter Bob Saget, a
board member of the SRF, directed the 1996 ABC TV movie For Hope, starring Dana
Delany, which depicts a young woman fatally affected by scleroderma; the film was
based on the experiences of Saget's sister Gay.[39]

[edit] References
1. ^ Scleroderma at Dorland's Medical Dictionary
2. ^ MeSH Systemic+Scleroderma
3. ^ MeSH Scleroderma,+Localized
4. ^ a b c d e Klippel, John H.. Primer On the Rheumatic Diseases 11ED. Atlanta, GA:
Arthritis Foundation. ISBN 1-912423-16-2.
5. ^ Valentini G, Black C (2002). "Systemic sclerosis". Best practice & research.
Clinical rheumatology 16 (5): 807–16. doi:10.1053/berh.2002.0258. PMID
12473275.
6. ^ Olsen NJ, King LE, Park JH (1996). "Muscle abnormalities in scleroderma".
Rheum. Dis. Clin. North Am. 22 (4): 783–96. doi:10.1016/S0889-857X(05)70301-
X. PMID 8923596.
7. ^ Steen VD (2005). "The lung in systemic sclerosis". Journal of clinical
rheumatology 11 (1): 40–6. PMID 16357695.
8. ^ a b c Sallam H, McNearney TA, Chen JD (2006). "Systematic review:
pathophysiology and management of gastrointestinal dysmotility in systemic
sclerosis (scleroderma)". Aliment. Pharmacol. Ther. 23 (6): 691–712.
doi:10.1111/j.1365-2036.2006.02804.x. PMID 16556171.
9. ^ a b c Rose S, Young MA, Reynolds JC (1998). "Gastrointestinal manifestations of
scleroderma". Gastroenterol. Clin. North Am. 27 (3): 563–94. doi:10.1016/S0889-
8553(05)70021-2. PMID 9891698.
10. ^ Hendel L, Hage E, Hendel J, Stentoft P (1992). "Omeprazole in the long-term
treatment of severe gastro-oesophageal reflux disease in patients with systemic
sclerosis". Aliment. Pharmacol. Ther. 6 (5): 565–77. PMID 1420748.
11. ^ Ruangjutipopan S, Kasitanon N, Louthrenoo W, Sukitawut W, Wichainun R
(2002). "Causes of death and poor survival prognostic factors in thai patients with
systemic sclerosis". Journal of the Medical Association of Thailand 85 (11):
1204–9. PMID 12546318.
12. ^ Steen VD, Mayes MD, Merkel PA (2003). "Assessment of kidney
involvement". Clin. Exp. Rheumatol. 21 (3 Suppl 29): S29–31. PMID 12889219.
13. ^ Steen VD (1994). "Renal involvement in systemic sclerosis". Clin. Dermatol.
12 (2): 253–8. doi:10.1016/S0738-081X(94)90329-8. PMID 8076263.
14. ^ Steen VD (2003). "Scleroderma renal crisis". Rheum. Dis. Clin. North Am. 29
(2): 315–33. doi:10.1016/S0889-857X(03)00016-4. PMID 12841297.
15. ^ Rhew EY, Barr WG (2004). "Scleroderma renal crisis: new insights and
developments". Current rheumatology reports 6 (2): 129–36. doi:10.1007/s11926-
004-0057-5. PMID 15016343.
16. ^ a b Steen VD, Medsger TA (2000). "Long-term outcomes of scleroderma renal
crisis". Ann. Intern. Med. 133 (8): 600–3. PMID 11033587.
17. ^ a b c Jimenez S, Koenig AS. Scleroderma. eMedicine.com. Accessed: May 22,
2006.
18. ^ Penn H, Howie AJ, Kingdon EJ, et al (August 2007). "Scleroderma renal crisis:
patient characteristics and long-term outcomes". QJM 100 (8): 485–94.
doi:10.1093/qjmed/hcm052. PMID 17601770.
19. ^ Pham PT, Pham PC, Danovitch GM, et al (October 2005). "Predictors and risk
factors for recurrent scleroderma renal crisis in the kidney allograft: case report
and review of the literature". Am. J. Transplant. 5 (10): 2565–9.
doi:10.1111/j.1600-6143.2005.01035.x. PMID 16162209.
20. ^ a b c d e f g Jimenez SA, Derk CT (2004). "Following the molecular pathways
toward an understanding of the pathogenesis of systemic sclerosis". Ann. Intern.
Med. 140 (1): 37–50. PMID 14706971.
21. ^ "Preliminary criteria for the classification of systemic sclerosis (scleroderma).
Subcommittee for scleroderma criteria of the American Rheumatism Association
Diagnostic and Therapeutic Criteria Committee" (1980). Arthritis Rheum. 23 (5):
581–90. PMID 7378088. Available online at "1980 Criteria for the Classification
of Systemic Sclerosis". Retrieved on 2007-08-05.
22. ^ Winterbauer RH (1964). "Multiple telangiectasia, Raynaud'S phenomenon,
sclerodactyly, and subcutanious calcinosis: a syndrome mimicking hereditary
hemorrhagic telangiectasia". Bulletin of the Johns Hopkins Hospital 114: 361–83.
PMID 14171636.
23. ^ Morpea CNN.com, (May 05, 2006).
24. ^ Bianchi DW (2000). "Fetomaternal cell trafficking: a new cause of disease?".
Am. J. Med. Genet. 91 (1): 22–8. doi:10.1002/(SICI)1096-
8628(20000306)91:1<22::AID-AJMG4>3.0.CO;2-3. PMID 10751084.
25. ^ Galan A, Cowper SE, Bucala R (2006). "Nephrogenic systemic fibrosis
(nephrogenic fibrosing dermopathy)". Current opinion in rheumatology 18 (6):
614–7. doi:10.1097/01.bor.0000245725.94887.8d. PMID 17053507.
 26. ^ Boyd AS, Zic JA, Abraham JL (2007). "Gadolinium deposition in nephrogenic
fibrosing dermopathy". J. Am. Acad. Dermatol. 56 (1): 27–30.
doi:10.1016/j.jaad.2006.10.048. PMID 17109993.
27. ^ Sharma SK, Handa R, Sood R, et al (2004). "Bleomycin-induced scleroderma".
The Journal of the Association of Physicians of India 52: 76–7. PMID 15633728.
28. ^ Farrant PB, Mortimer PS, Gore M (2004). "Scleroderma and the taxanes. Is
there really a link?". Clin. Exp. Dermatol. 29 (4): 360–2. doi:10.1111/j.1365-
2230.2004.01519.x. PMID 15245529.
29. ^ Kettaneh A, Al Moufti O, Tiev KP, et al (2007). "Occupational exposure to
solvents and gender-related risk of systemic sclerosis: a metaanalysis of case-
control studies". J. Rheumatol. 34 (1): 97–103. PMID 17117485.
30. ^ Fonseca C, Lindahl GE, Ponticos M, et al (September 2007). "A polymorphism
in the CTGF promoter region associated with systemic sclerosis". N. Engl. J.
Med. 357 (12): 1210–20. doi:10.1056/NEJMoa067655. PMID 17881752.
31. ^ Oliver GF, Winkelmann RK (1989). "The current treatment of scleroderma".
Drugs 37 (1): 87–96. PMID 2651089.
32. ^ a b c d e Zandman-Goddard G, Tweezer-Zaks N, Shoenfeld Y (2005). "New
therapeutic strategies for systemic sclerosis--a critical analysis of the literature".
Clin. Dev. Immunol. 12 (3): 165–73. PMID 16295521. Full text at PMC: 2275417
33. ^ Tashkin DP, Elashoff R, Clements PJ, et al (June 2006). "Cyclophosphamide
versus placebo in scleroderma lung disease". N. Engl. J. Med. 354 (25): 2655–66.
doi:10.1056/NEJMoa055120. PMID 16790698.
34. ^ Hoyles RK, Ellis RW, Wellsbury J, et al (December 2006). "A multicenter,
prospective, randomized, double-blind, placebo-controlled trial of corticosteroids
and intravenous cyclophosphamide followed by oral azathioprine for the
treatment of pulmonary fibrosis in scleroderma". Arthritis Rheum. 54 (12): 3962–
70. doi:10.1002/art.22204. PMID 17133610.
35. ^ Nash RA, McSweeney PA, Crofford LJ, et al (2007). "High-dose
immunosuppressive therapy and autologous hematopoietic cell transplantation for
severe systemic sclerosis: long-term follow-up of the U.S. multicenter pilot
study". Blood 110 (4): 1388–96. doi:10.1182/blood-2007-02-072389. PMID
17452515.
36. ^ "Juvenile Scleroderma Network". Retrieved on 2008-05-11.
37. ^ "Scleroderma Foundation". Retrieved on 2008-05-11.
38. ^ "Scleroderma Research Foundation". Retrieved on 2008-05-11..
39. ^ Scleroderma at the Internet Movie Database

[edit] External links

• DermnetNZ: Systemic sclerosis
• Juvenile Scleroderma Network
• Juvenile Systemic Sclerosis
• Scleroderma Foundation
• Scleroderma Society of Ontario
• International Scleroderma Network
• The Scleroderma Research Foundation
• UK Scleroderma Society
• Scleroderma Information from Johns Hopkins University
• Scleroderma Australia
 [show]
v•d•e
Diseases of the skin and appendages by morphology

verruca, clavus, seborrheic keratosis, acrochordon, molluscum

Epidermal contagiosum, actinic keratosis, squamous cell carcinoma, basal cell
carcinoma, merkel cell carcinoma, nevus sebaceous, trichoepithelioma

Pigmented ephelis, lentigo, melasma, nevus, malignant melanoma

epidermal inclusion cyst, hemangioma, dermatofibroma, keloid,

lipoma, neurofibroma, xanthoma, Kaposi's sarcoma, infantile digital
Dermal and subcutaneous
fibromatosis, granular cell tumor, leiomyoma, lymphangioma
circumscriptum, myxoid cyst

essential dermatitis, contact dermatitis, atopic

dermatitis, seborrheic dermatitis, stasis
dermatitis, lichen simplex chronicus, Darier's
Eczematous disease, glucagonoma syndrome, langerhans
cell histiocytosis, lichen sclerosus, pemphigus
foliaceus, Wiskott-Aldrich syndrome, Zinc
deficiency

psoriasis, tinea corporis, tinea cruris, tinea

pedis, tinea manuum, tinea faciale, pityriasis
Scaling rosea, secondary syphillis, mycosis fungoides,
systemic lupus erythematosus, pityriasis rubra
pilaris, parapsoriasis, icthyosis

herpes simplex, herpes zoster, varicella, bullous

impetigo, acute contact dermatitis, pemphigus
Blistering vulgaris, bullous pemphigoid, dermatitis
With epidermal involvement herpetiformis, porphyria cutanea tarda,
epidermolysis bullosa simplex

scabies, insect bite reactions, lichen planus,

miliaria, keratosis pilaris, lichen spinulosus,
Papular transient acantholytic dermatosis, lichen
nitidus, pityriasis lichenoides et varioliformis
acuta

acne vulgaris, acne rosacea, folliculitis,

impetigo, candidiasis, gonococcemia,
Pustular
dermatophyte, coccidioidomycosis, subcorneal
pustular dermatosis
 [show]
v•d•e
Diseases of the skin and subcutaneous tissue (integumentary system) (L,
680-709)

Atopic dermatitis - Seborrhoeic dermatitis (Dandruff,

Cradle cap) - Contact dermatitis (Diaper rash,
Urushiol-induced contact dermatitis) - Erythroderma -
Dermatitis and eczema
Lichen simplex chronicus/Prurigo nodularis - Itch
(Pruritus ani) - Nummular dermatitis - Dyshidrosis -
Pityriasis alba

Psoriasis (Psoriatic arthritis) - Parapsoriasis (Pityriasis

lichenoides et varioliformis acuta, Pityriasis
lichenoides chronica, Lymphomatoid papulosis) -
Papulosquamous disorders
other pityriasis (Pityriasis rosea, Pityriasis rubra
pilaris) - other lichenoid (Lichen planus, Lichen
nitidus)

Dermatographic urticaria - Cold urticaria - Cholinergic

Urticaria
urticaria

Erythema multiforme/drug eruptions: Stevens-Johnson

syndrome - Toxic epidermal necrolysis - Erythema
nodosum
Erythema
Other erythema: Erythema annulare centrifugum -
Erythema marginatum - Necrolytic migratory erythema
- Erythema toxicum

Ingrown nail - Onychogryposis - Beau's lines - Yellow nail syndrome

Nail
- Leukonychia

Alopecia areata (Alopecia totalis, Alopecia universalis,

Ophiasis)
Hair lossAndrogenic alopecia - Hypotrichosis - Telogen effluvium -
Traction alopecia - Lichen planopilaris - Trichorrhexis
nodosa

Hypertrichosis (Hirsutism)

Acneiform eruption (Acne vulgaris, Chloracne, Blackhead)

 [show]
v•d•e
Systemic CT disorders (M30-M36, 710, 440-448)

Large vesselTakayasu's arteritis · Temporal arteritis

Medium vesselPolyarteritis nodosa · Wegener's granulomatosis · Kawasaki disease

Churg-Strauss syndrome · Microscopic polyangiitis ·

Small vessel
Hypersensitivity vasculitis

Retrieved from "http://en.wikipedia.org/wiki/Scleroderma"

Categories: Autoimmune diseases | Diseases involving the fasciae | Rheumatology
Hidden categories: All articles with unsourced statements | Articles with unsourced
statements since September 2008 | Articles with unsourced statements since May 2008 |
Articles with limited geographic scope

Views

• Article
• Discussion
• Edit this page
• History

Personal tools

• Log in / create account

Navigation

• Main page
• Contents
• Featured content
• Current events
• Random article

Search

Interaction
 • About Wikipedia
• Community portal
• Recent changes
• Contact Wikipedia
• Donate to Wikipedia
• Help

Toolbox

• What links here

• Related changes
• Upload file
• Special pages
• Printable version
• Permanent link
• Cite this page

Languages

• Български
• Deutsch
• Español
• Français
• Italiano
• ‫עברית‬
• Nederlands
• 日本語
• Polski
• Português
• Slovenčina
• Svenska
• 中文

• This page was last modified on 25 October 2008, at 13:28.

• All text is available under the terms of the GNU Free Documentation License.
(See Copyrights for details.)
Wikipedia® is a registered trademark of the Wikimedia Foundation, Inc., a U.S.
registered 501(c)(3) tax-deductible nonprofit charity.
• Privacy policy
• About Wikipedia
• Disclaimers