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Systemic Sclerosis: Diffuse Scleroderma

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
What is Diffuse Scleroderma?
Causes of Scleroderma
Diagnosis
Sine: Diffuse Without Skin Involvement
Complications
Progression
Treatment
References
Diffuse Patient Stories

What is Diffuse Scleroderma?


Overview of Diffuse Systemic Scleroderma
Presented by Amanda Thorpe

Diffuse Systemic Scleroderma is also variously known as Scleroderma (SD), Diffuse Sclerosis, Diffuse Scleroderma, Diffuse Cutaneous Scleroderma, Systemic Sclerosis (SSc), Progressive Systemic Sclerosis (PSS).

It also includes Scleroderma sine Scleroderma (Scleroderma without Scleroderma), and the subset of Familial Progressive Systemic Sclerosis (FPSS). (Also see: What is Scleroderma? and Types of Scleroderma)

Causes of Scleroderma

Causes of Scleroderma. The cause of scleroderma is generally unknown, however areas being investigated include autoimmunity, environmental exposures, genetics, and infections. Scleroderma is not believed to be contagious; you cannot get it by hugging or kissing someone or other intimate contact. However there is an increased incidence of certain types of cancer among scleroderma patients. (Also see: Associated Conditions) ISN.

Diagnosis

Diffuse Scleroderma is diagnosed whenever there is proximal tight skin. Proximal means located closest to the reference point. And here is where the plot thickens for Scleroderma patients who have skin tightness only between their wrists and their elbows, because there are two different definitions of what the reference point should be when bandying about this proximal word:
1. The American College of Rheumatology defines proximal tight skin as skin tightness above the wrists. (1)
2. Dr. LeRoy defines proximal tight skin as skin tightness above the elbows. Europeans and many centers in the United States use this definition for proximal tight skin. (2)
Therefore, someone with skin tightness only between their elbows and their wrists will receive a diagnosis of either Diffuse or Limited Systemic Scleroderma, depending on which definition of "proximal" their doctor uses.
The diagnosis is clinical, and requires no laboratory or special testing.

Sine: Diffuse Scleroderma Without Skin Involvement

"Skin sclerosis is no longer regarded as mandatory for the diagnosis of systemic sclerosis." Prof. Carol Black, 2004
Sometimes Diffuse Systemic Scleroderma leaves the skin and joints untouched, and affects only the connective tissue of the digestive system or some other internal body system. In the absence of visible skin involvement, "Scleroderma sine Scleroderma," is diagnosed, which basically means "Diffuse Scleroderma without Scleroderma (skin involvement)."
In Scleroderma sans Scleroderma, Raynaud's may or may not be present. The usual presentation is with pulmonary (lung) fibrosis, and/or Scleroderma renal (kidney) crisis, and/or cardiac (heart) disease, and/or gastrointestinal disease. Antinuclear antibodies (ANA) may sometimes (but not necessarily) be present (Scl-70, ACA.)
Systemic sclerosis sine scleroderma (ssSSc) and limited cutaneous systemic sclerosis (lcSSc): Similarities and differences. ssSSc and lcSSc patients share demographic, clinical and immunologic features although differences are mainly due to peripheral vascular manifestations. ssSSc patients should be truly differentiated from early SSc using sensitive and specific studies looking for any asymptomatic organ involvement. PubMed, Clin Exp Rheumatol. 2014 Apr 28. (Also see: Limited Scleroderma)
Systemic sclerosis sine scleroderma associated with antiphospholipid syndrome (APS). Cases described in medical literature associate the presence of antiphospholipid antibodies with the presence of APS and systemic sclerosis (SS). We describe the first case of a patient with APS associated with systemic sclerosis sine scleroderma. Rheumatol Int. 2012 Oct;32(10):3265-8.
Systemic sclerosis sine scleroderma: distinct features in a large Brazilian cohort. Among the 947 consecutive patients with SSc, 79 (8.3%) were classified as ssSSc. Oesophagus was the most frequently affected organ (83.1%), followed by pulmonary involvement (63.2%). Oxford Journals. August 2012.

Complications

Diffuse Systemic Scleroderma causes variety of complications that can affect the skin, mouth, eyes and internal organs such as the lungs, heart, kidneys, and the entire gastrointestinal tract. See Scleroderma Symptoms and Treatments.

Progression

About 95 percent of people with Diffuse Systemic Scleroderma show some early thickening and hardening of the skin, especially of the hands, arms and/or face. This is due to the increased production of fibrous scar tissue. Widespread skin involvement more often results in severe internal organ damage than in patients with less skin involvement.
Usually, the skin on the fingertips tightens first, then progresses to the fingers, hands, forearms, and upper arms. By the time the skin of the arms is tight, there may be stiffness of the legs, thighs, and in some cases, chest and abdomen. The skin tightness usually appears on both sides of the body and is symmetrical. The skin of the face and neck may also become involved. See Scleroderma Photos.

Treatments and Clinical Trials

Scleroderma Treatments and Clinical Trials. ISN.

References

(1) Paper for ACR criteria: Subcommittee for Scleroderma criteria of the American Rheumatism Association diagnostic and therapeutic criteria committee. 1980. Preliminary criteria for the classification of systemic sclerosis (Scleroderma). Arthritis Rheum. 23,581:590.
(2) Paper for LeRoy's criteria: Kahaleh MB, Sultany GL, Loadholt CB, Smith EA, Huffstutter JE, and LeRoy EC. 1986. A modified Scleroderma skin scoring method. Clinical & Experimental Rheumatology. (4):367-369.

Diffuse Scleroderma Patient & Caregiver Stories

Amanda: Diffuse Scleroderma Systemic Sclerosis I am thirty-nine years old and was diagnosed with diffuse scleroderma systemic sclerosis in August 2007...
Ana Lucia: Diffuse Scleroderma I am twenty years old and I was diagnosed with diffuse systemic scleroderma a year and a half ago...
(Español/Spanish) Ana Lucia: Esclerosis Sistemica Difusa Estoy en tratamiento de corticoides. Y ciclofosfamida llevo un año y medio en hospitales he tenido que ingresar...
Anastasia: Surviving Granddaughter of a Diffuse Scleroderma Patient We went everywhere to figure out what she had but no one knew. Traveling to a different hospital finally we heard scleroderma...
More Diffuse Scleroderma Stories...
 
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