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Collagen vascular disease

In a class of diseases known as autoimmune disorders, the body's immune system attacks its own tissues. Some of these diseases are similar to each other. They may involve arthritis and inflammation of arteries in the tissues. People who developed these disorders were previously said to have "connective tissue" or "collagen vascular" disease. We now have names for many specific conditions such as:

When a specific disease cannot be diagnosed, more general terms may be used. These are called undifferentiated systemic rheumatic (connective tissue) diseases or overlap syndromes.

References

Bennett RM. Overlap syndromes. In: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, eds. Kelley and Firestein's Textbook of Rheumatology. 10th ed. Philadelphia, PA: Elsevier; 2017:chap 86.

Mims MP. Lymphocytosis, lymphocytopenia, hypergammaglobulinemia, and hypogammaglobulinemia. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 7th ed. Philadelphia, PA: Elsevier; 2018:chap 49.

  • Dermatomyositis - heliotrope eyelids

    Dermatomyositis - heliotrope eyelids - illustration

    This photograph demonstrates the sign heliotrope eyelids in which the eyelids develop a brown (violaceous - rather than red) color. Heliotrope eyelids and Gottron's papules on the knuckles are characteristic findings in dermatomyositis.

    Dermatomyositis - heliotrope eyelids

    illustration

  • Polyarteritis - microscopic on the shin

    Polyarteritis - microscopic on the shin - illustration

    This is a picture of microscopic polyarteritis on the shin. The term polyarteritis means that many blood vessels are inflamed. These nodules are located just below the skin's surface (subcutaneous), are barely felt when pressing on the skin (palpable), and are tender. The skin is typically red (erythematous).

    Polyarteritis - microscopic on the shin

    illustration

  • Systemic lupus erythematosus rash on the face

    Systemic lupus erythematosus rash on the face - illustration

    This is a photo of a systemic lupus erythematosis rash on the face. Lupus erythematosis often produces a butterfly rash or malar rash. Typically, the rash also appears on the nose.

    Systemic lupus erythematosus rash on the face

    illustration

  • Sclerodactyly

    Sclerodactyly - illustration

    The most classic symptom of scleroderma is a type of skin tightening called sclerodactyly. The initial stages of the disease involves swelling of the fingers. Later, as the connective tissue becomes fibrotic, skin on the fingers and toes becomes hard and shiny. The fingers can become difficult to bend and can form contractures due to the severe tightening of the skin.

    Sclerodactyly

    illustration

  • Rheumatoid arthritis

    Rheumatoid arthritis - illustration

    The affect of rheumatoid arthritis can progress to the degree that it is crippling. Deformities distinctive to late-stage rheumatoid arthritis such as ulnar deviation of the bones of the hands, or swan-neck deviation of the fingers occur because muscles and tendons on one side of the joint may overpower those on the other side, pulling the bones out of alignment.

    Rheumatoid arthritis

    illustration

    • Dermatomyositis - heliotrope eyelids

      Dermatomyositis - heliotrope eyelids - illustration

      This photograph demonstrates the sign heliotrope eyelids in which the eyelids develop a brown (violaceous - rather than red) color. Heliotrope eyelids and Gottron's papules on the knuckles are characteristic findings in dermatomyositis.

      Dermatomyositis - heliotrope eyelids

      illustration

    • Polyarteritis - microscopic on the shin

      Polyarteritis - microscopic on the shin - illustration

      This is a picture of microscopic polyarteritis on the shin. The term polyarteritis means that many blood vessels are inflamed. These nodules are located just below the skin's surface (subcutaneous), are barely felt when pressing on the skin (palpable), and are tender. The skin is typically red (erythematous).

      Polyarteritis - microscopic on the shin

      illustration

    • Systemic lupus erythematosus rash on the face

      Systemic lupus erythematosus rash on the face - illustration

      This is a photo of a systemic lupus erythematosis rash on the face. Lupus erythematosis often produces a butterfly rash or malar rash. Typically, the rash also appears on the nose.

      Systemic lupus erythematosus rash on the face

      illustration

    • Sclerodactyly

      Sclerodactyly - illustration

      The most classic symptom of scleroderma is a type of skin tightening called sclerodactyly. The initial stages of the disease involves swelling of the fingers. Later, as the connective tissue becomes fibrotic, skin on the fingers and toes becomes hard and shiny. The fingers can become difficult to bend and can form contractures due to the severe tightening of the skin.

      Sclerodactyly

      illustration

    • Rheumatoid arthritis

      Rheumatoid arthritis - illustration

      The affect of rheumatoid arthritis can progress to the degree that it is crippling. Deformities distinctive to late-stage rheumatoid arthritis such as ulnar deviation of the bones of the hands, or swan-neck deviation of the fingers occur because muscles and tendons on one side of the joint may overpower those on the other side, pulling the bones out of alignment.

      Rheumatoid arthritis

      illustration

    A Closer Look

     

    Tests for Collagen vascular disease

     

     

    Review Date: 1/10/2019

    Reviewed By: Gordon A. Starkebaum, MD, MACR, ABIM Board Certified in Rheumatology, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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