Scleroderma (With Pictures)- Symptoms and It's Treatment
Alternative names :- CREST syndrome; Progressive systemic sclerosis; Systemic Sclerosis
Scleroderma literally means "hard skin." It's an uncommon disorder of diffuse connective tissue disease characterized by inflammatory and then degenerative and fibrotic changes (hardening and scarring) in the skin, blood vessels, synovial membranes, skeletal muscles, and internal organs (especially the esophagus, intestinal tract, thyroid, heart, lungs, and kidneys). There are two major types of scleroderma -localized and systemic - and each type has many subtypes.
Localized scleroderma usually affects only the skin. It has three main subtypes: morphea, generalized morphea, and linear. Systemic scleroderma affects multiple systems, including blood vessels, internal organs, muscles, joints, and the intestines. It's divided into two subtypes: systemic limited scleroderma (also known as CREST) and systemic-diffuse. There's also a type called sclerodenna sine sclerosis, which affects the internal organs but doesn't have the characteristic skin ulcers of systemic scleroderma.
Scleroderma occurs in 3 to 4 times as many women as men, especially those between ages 30 and 50. The peak incidence of occurrence is in women ages 50 to 60. Scleroderma usually progresses slowly. When the condition is limited to the skin, the
prognosis is usually favorable. However, approximately 30% of patients with systemic scleroderma die within 5 years of onset. Death is usually caused by infection or renal or heart failure.
What causes Scleroderma?
The cause of sclerodenna is unknown, but some possible causes include:
- systemic exposure to silica dust or polyvinyl chloride
- anticancer agents, such as bleomycin (Blenoxane), or nonopioid analgesics such as pentazocine (Talwin)
- fibrosis due to an abnormal immune system response
- underlying vascular cause with tissue changes initiated by a persistent perfusion. For example, in some patients, muscles and joints become fibrotic.
Who gets scleroderma
|Scleroderma is a relatively rare illness affecting only 75,000 - 100,000 people in the United States. Of these, 75% percent are women usually diagnosed between the ages of 30 and 50 years. Twins and family members of patients with scleroderma or other autoimmune connective tissue diseases, such as systemic lupus erythematosus , appear to be at a slightly increased risk. Children can get scleroderma, although the pattern and extent of disease may be different in children.
Signs and symptoms of Scleroderma
Scleroderma is characterized by diffuse fibrosis, degenerative changes, and vascular changes in the skin, particular structures, and internal organs. It usually begins in the fingers and extends proximally to the upper arms, shoulders, neck, and face. The skin atrophies, edema and infiltrates containing CD4 + T cells surround the blood vessels, and inflamed collagen fibers become edematous, losing strength and elasticity. The dermis becomes tightly bound to the underlying structures, resulting in atrophy of the affected dermal appendages and destruction of the distal phalanges by osteoporosis. As the disease progresses. this atrophy can affect other areas.
Possible signs and symptoms of scleroderma include:
- skin thickening, commonly limited to the distal extremities and face but possibly involving internal organs (systemic-limited scleroderma)
- CREST syndrome (calcinosis, Raynaud's phenomenon. esophageal dysfunction. sclerodactyly, and telangiectasia), a benign subtype of systemic limited scleroderma
- generalized skin thickening and involvement of internal organs (systemic-diffuse scleroderma)
- patchy skin changes with a teardrop shape known as morphea localized scleroderma)
- band of thickened skin on the face or extremities that severely damages underlying tissues, causing atrophy and deformity localized linear scleroderma)
- atrophy and deformity (most common in childhood)
- Raynaud's phenomenon (blanching, cyanosis, and erythema of the fingers and toes when exposed to cold or stress) and progressive phalangeal resorption, which may shorten the fingers (early symptoms)
- pain, stiffness, and swelling of fingers and joints Gater symptoms)
- Sclerodactyly (taut, shiny skin over the entire hand and forearm due to skin thickening)
- tight and inelastic facial skin causing a mask like appearance and "pinching" of the mouth; contractures with progressive tightening
- thickened skin over proximal limbs and trunk (systemic-diffuse scleroderma)
- painful ulcers (systemic-limited and systemic-diffuse scleroderma)
- frequent reflux, heartburn, dysphagia, and bloating after meals due to GI dysfunction
- abdominal distention, diarrhea, constipation, and malodorous floating stool.
Complications of scleroderma include compromised circulation due to abnormal thickening of the arterial intima, possibly causing slowly healing ulcerations on fingertips or toes leading to gangrene; decreased food intake and weight loss due to GI symptoms; and arrhythmias and dyspnea due to cardiac and pulmonary fibrosis and renal crisis (malignant hypertension due to renal involvement), which may be fatal if untreated (advanced disease).
Tests to aid diagnosis of scleroderma may include:
- typical cutaneous changes (the first clue to diagnosis)
- slightly elevated erythrocyte sedimentation rate, positive rheumatoid factor in 25% to 35% of patients, and positive antinuclear antibody test results
- urinalysis showing proteinuria, microscopic hematuria and casts (with renal involvement)
- hand X-rays showing terminal phalangeal tuft resorption, subcutaneous calcification, and joint space narrowing and erosion
- chest X-rays showing bilateral basilar pulmonary fibrosis
- Gl X-rays showing distal esophageal hypomotility and stricture, duodenalloop dilation, small-bowel malabsorption pattern, and large diverticula
- pulmonary function studies showing decreased diffusion and vital capacity
- electrocardiogram showing nonspecific abnormalities related to myocardial fibrosis
- skin biopsy showing changes consistent with disease progression. such as marked thickening of the dennis and occlusive vessel changes.
Treatment of Scleroderma
There's no cure for scleroderma. Treatment aims to preserve normal body functions, minimize complications and may include:
- immunosuppressants, including such common palliative drugs as cyclosporine (Neoral) and chlorambucil (Leukeran)
- vasodilators and antihypertensives, such as nifedipine (Adalat), prazosin (Minipress), or topical nitroglycerin Nitro-Bid); digital sympathectomy; or, rarely, cervical sympathetic blockade to treat Raynaud's phenomenon, digital plaster cast to immobilize the area, minimize trauma, and maintain cleanliness; possible surgical debridement for chronic digital ulceration
- antacids to reduce total add level in GI tract; omeprazole (Prilosec), a proton-pump inhibitor to block the formation of gastric add, periodic dilation, and a soft, bland diet for esophagitis with stricture
- broad-spectrum antibiotics to treat small-bowel involvement with erythromycin or tetracycline (preferred drugs) to counteract the bacterial overgrowth in the duodenum and jejunum related to hypomotility
- short-term benefit from vasodilators, such as nifedipine (Adalat) and hydralazine (Apresoline), to decrease contractility and oxygen demand and cause vasodilation (for pulmonary hypertension)
- angiotensin-converting enzyme (ACE) inhibitor to preserve renal function (early intervention in renal crisis)
- physical therapy to maintain function and promote muscle strength, heat therapy to relieve joint stiffness and occupational therapy to help performance of daily activities easier (for hand debilitation).
Special considerations and Prevention
- Assess motion restrictions, pain, vital signs, intake and output, respiratory function, and daily weight.
- Because of compromised circulation, warn against fingers tick blood tests.
- Remember that air conditioning may aggravate Raynaud's phenomenon.
- Help the patient and her family adjust to the patient's new body image and the limitations and dependence that these changes cause.
- Teach the patient to avoid fatigue by pacing activities and organizing schedules to include necessary rest.
- Scleroderma differs from person to person, but can be a very serious disease.
- The patient and family need to accept the fact that this condition is incurable. Encourage them to express their feelings and help them cope with their fears and frustrations by offering information about the disease.its treatment. and relevant diagnostic tests.
- Whenever possible, let the patient participate in treatment by measuring her own intake and output, planning her own diet, giving herself heat therapy, and performing prescribed exercises independently.