Scleroderma is a multi-system disease characterized by functional and structural abnormalities of small blood vessels, thickening of the skin and internal organs, and immune system activation. It is an acquired, non-contagious, rare disease of unknown etiology that occurs sporadically worldwide.
Scleroderma is classified into several subsets defined by the degree of clinically involved skin. These subsets include:
Diffuse Cutaneous Scleroderma
- Proximal skin thickening involving face/neck, trunk and symmetrically, the fingers, arms, and legs
- Rapid onset of disease following the appearance of Raynaud’s phenomenon
- Significant visceral disease: lung, heart, gastrointestinal or kidney
- Associated with antinuclear antibodies and absence of anticentromere antibody
- Variable disease course but overall poor prognosis
Limited Cutaneous Scleroderma
- Skin thickening limited to a symmetrical change of fingers, distal arms, legs and face/neck
- Progression of disease after onset of Raynaud’s phenomenon
- Late visceral disease with prominent hypertension and digital amputation
- CREST syndrome (Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia)
- Association with anticentromere antibody
- Relatively good prognosis
Overlap Syndrome
- Diffuse or limited scleroderma with typical features of one or more of the other connective tissue diseases
- Mixed connective-tissue disease: features of systemic lupus erythematosus, scleroderma, polymyositis, rheumatoid arthritis, and presence of anti-RNP
Undefined Connective-Tissue Disease
- Patients with features of scleroderma who do not have definite clinical or laboratory findings to make a diagnosis
Localized Scleroderma
- Morphea: plaques of fibrotic skin and subcutaneous tissue without systemic disease
- Linear scleroderma: longitudinal fibrotic bands that occur predominantly on extremities and involve the skin and deeper tissues
Who Is At Risk?
Age, gender, and genetic background can play a big role in the development of scleroderma. Typically, this disease occurs in individuals between 35-65 years of age. Female predominance is most pronounced during the mid- and late-childbearing years.
What Are the Symptoms of Scleroderma?
The initial symptoms are typically nonspecific and include fatigue, musculoskeletal pain, and Raynaud’s phenomenon (cold hands and feet associated with color changes of the skin of the digits). These symptoms may persist for weeks or months before other signs emerge. The first specific clinical clue to suggest a diagnosis is skin thickening that begins as swelling or “puffiness” of the fingers and hands.
Raynaud’s phenomenon is one of the most common symptoms of patients with scleroderma. Symptoms appear suddenly as attacks are triggered by cold temperatures or emotional stress. Classic Raynaud’s phenomenon goes through three color changes, white, blue and red, although these changes do not occur in all cases. A reversal of the attack generally occurs in 10-15 minutes after the stimulus has ended.
Other organs, besides the skin and musculoskeletal system, can be affected by scleroderma as well. The lungs may be affected, leading to shortness of breath on exertion. Gastrointestinal tract involvement can present with difficulty swallowing, heartburn, or constipation. Cardiac manifestations may be subtle and usually occur late in the course of the disease. Finally, renal involvement may present with accelerated hypertension or acute renal failure.
How Is Scleroderma Treated?
Scleroderma has a wide spectrum of clinical manifestations and severity, as well as a variable course. Due to this, spontaneous improvements in the disease occur frequently. For treatment, therapy may be effective in helping to manage symptoms. Therapy can be divided into two categories: disease-modifying and symptomatic (organ-specific). It is very important to monitor and treat patients to try and slow down the progression of the disease.
Treatment for Scleroderma In Thousand Oaks, CA
If you have or think you may have scleroderma, there is help available. Offering his patients the most up to date and efficacious treatments, Dr. Cohen and his talented team at Cohen Medical Centers are ready to help you feel like yourself again. To learn more about treatment in Ventura County, simply call us at (805) 449-8781 to schedule your consultation; you may also fill out the form below, and a member of our helpful staff will get back to you.