SCLERODERMA

Systemic sclerosis scleroderma

Systemic sclerosis SSc (also known as scleroderma) literally

hard (scleros) skin (derma), encompasses both a disease
restricted to the skin (localized scleroderma) and a disease with
the involvement of internal organ (diffuse scleroderma or systemic
sclerodema)

Hardening of the skin

The term scleroderma was first introduced in mid-19th century to

describe skin induration

Occurs as a multisystem inflammatory disease characterized by

skin thickening and deposition of excessive quantities of
connective tissue and results in severe fibrosis

Incidence for women is 3x higher than for men

Scleroderma occurs between ages 30 to 50 years

What causes scleroderma is unknown

Several risk factors associated with SSc play a role:

o

Over production of collagen

Fibrous skin thickening asso. with environmental factors (working with

plastics, coal, silica dust)

High alcohol intake

Sclerodema-like conditions can also be present as a result of genetic

factors, metabolic disorders, malignancies, post infectious disorders
and neurologic conditions

Pathophysiology
SSc is the result from excessive production of fibroblasts. Lymphocytes
accumulate in the lower dermis of the skin that generate lymphokines
which stimulate fibroblast to produce excessive procollagen. After
secretion, it undergoes cross-linking to produce mature insoluble collagen.
Because of fibrotic changes, skin loss its elasticity and movement. There
is vascular changes in the development of SSc. When vascular
endothelium is injured, damaged blood vessels release vasoactive
substances which are stimulated to overproduce collagen. Proliferation of
the subintimal connective tissue results, along with fibrous thickening and
narrowing of lumina, thus leading to tissue ischemia.

Two major forms of Scleroderma

1.

Systemic Scleroderma
involvement

leads

to

some

internal

organ

2.

Localized Scleroderma only affects the skin without any

internal organ involvement; often appears in waxy form of
patches; usually comes on gradually

Types of Systemic scleroderma

a. Diffuse cutaneous scleroderma skin thickening on the trunk, face,
proximal and distal extremities
b. Limited cutaneous scleroderma characterized by skin changes
that are usually confined to fingers and distal portions of extremities
and face

Types of Localized scleroderma

a. Plaque morphea - oval patches that can range from 2 to 20 cm
across; surface is smooth, shiny and hairless; start off mauve in color,
then gradually become white. Older patches may become brown
b. Superficial morphea - similar to the plaque type; typically seen as
symmetrical mauve-colored patches; usually in skin folds of the groin,
armpits or under breasts

c. Linear scleroderma - occurs on an arm or leg of a child; a long,

narrow area of thickened skin

en coup de sabre - a deep form of linear scleroderma affecting the

scalp and temple; hair is lost over the affected skin, and the skull
bone may be shrunk underneath it

d. Generalized morphea - a rare type where there are more plaques

spread around different areas of the body

Systemic scleroderma

Clinical manifestations

Systemic scleroderma

Early: Arthralgia (joint pain) and Raynauds phenomenon (generally

bilateral vasospastic condition

Tricolor changes affecting fingers: pallor (white) accompanying

vasoconstriction followed by cyanosis (blue) as capillary blood is
desaturated of O2 followed by hyperemia (redness) secondary to
vasodilation

Progressive skin tightness and induration, often preceded by swelling

and puffiness (edematous stage) that does not respond to diuretic
therapy

Prominent skin pigmentary changes both hyperpigmentation and

hypopigmentation

Chest pain (precordial) due to pulmonary artery hypertension

Loss in joint range of motion and joint flexion contractures

Hypertension

GI

Cardiovascular

GERD

- decreased blood flow

Dysphagia

- digit necrosis: severe

Small sliding hiatal hernia

- excruciating pain: severe

Constipation

- myocardial fibrosis: common

Malabsorption common

- cardiac dysrhythmias

Abdominal cramping

- chest pain

Pulmonary system

Renal

Pulmonary arterial hypertension

- Proteinuria

Exertional dyspnea

- Hematuria
- Hypertension
- Renal failure

Diffuse cutaneous scleroderma

Begins with symmetrical widespread thickening of the skin and

extremities

Early stage: bilateral symmetrical swelling of the fingers, face, feet

and skin has tense, wrinkle-free appearance

Disease progression: skin becomes more thickened, hidebound, and

shiny

Changes in pigmentation (hypopigmentation & hyperpigmentation)

are asso. With loss of skin folds

Distal thickening is always more severe than proximal thickening

Face become mask-like

Mouth is rigid, and overall expression is blunted or immobile

Has tendency to develop GI, heart, lungs and kidney problems

Rapid progression

Limited cutaneous scleroderma

Progresses much slowly than diffuse

Truncal scleroderma is absent

Visceral changes (e.g., severe pulmonary arterial hypertension and

biliary cirrhosis)

Often develop CREST

 Often develop painful ulcers

on the finger tips or in areas
of calcinosis because of
chronic vascular insufficiency

Localized scleroderma

Discolored patches

Thick, hard skin on arms and legs

Diagnostic tests

No single test is specific for systemic sclerosis. The following tests

may be ordered: [Lemone p. 1276]

Erythrocyte sedimentation rate (ESR) (blood) typically elevated

Blood tests

Magnetic Resonance Imaging (MRI) scan

Skin biopsy

Antinuclear antibody test (ANA)

ECG

Treatment
Medications

Immunosuppressive agents and corticosteroids may be used to

slow or prevent pulmonary fibrosis

Penicillamine may be used to treat scleroderma and pulmonary

fibrosis

Clacium hcannel blockers [nifedipine (Procardia)] or alphaadrenergic blockers [prazosin (Minipress)] - prescribed for Raynauds
phenomenon

H2-receptor blockers [cimetidine (Tagamet)/ ranitidine (Zantac)];

antacids, or omeprazole for esophagitis

Tetracycline or other broad-spectrum antibiotic relieve symptoms of

malabsorption

ACE inhibitors [captopril (Capoten)] to control hypertension

Dialysis and kidney transplant (surgical) end-stage kidney disease

Nursing interventions

Teach importance of avoiding cold and protecting fingers with mittens in cold
weather and when shopping in frozen-food section

Wear warm socks and properly fitting shoes

Maintain core temperature, always dress warmly

Frequent inspection for early ulcers

Smoking cessation

Apply moisturizers to prevent dryness and cracking

Perform facial exercises

Keep in a sitting or Fowlers position after meals

Elevate head of the bed at night

Encourage client to share concerns with family members

Move or reposition to minimize discomfort

Perform ROM exercises

Provide small, frequent meals (meals that are easy to swallow, high-calorie snack)

Avoid heavy snacks

Eat high fiber foods and drink more fluids

References
http://www.patient.co.uk/health/localised-scleroderma-morphoea
http://www.clevelandclinicmeded.com/medicalpubs/diseasemanageme
nt/rheumatology/systemic-sclerosis/#
s0015
http://emedicine.medscape.com/article/331864-clinical
Medical-Surgical Nursing [Brunner and Suddarths; Lemone; Joyce
Black; and Ignatavicius 5th edition]