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]
