Scleroderma

Scleroderma is derived from the Greek words skleros (hard or indurated) and derma (skin). Hippocrates first described this condition as thickened skin.^[1] Carlo Curzio (1752) offered the first detailed description of scleroderma in a patient with hard skin, which he described as woodlike or as containing a dry hide. In 1836, Giovambattista Fantonetti applied the term scleroderma to a patient's condition.^[2] He applied the term to describe a patient with dark leatherlike skin who exhibited a loss of range of joint motion due to skin tightening. Robert H. Goetz first described in detail the concept of scleroderma as a systemic disease in 1945; he introduced the term progressivesystemic sclerosis  to emphasize the systemic and often progressive nature of the disease.

Definition

The term systemic sclerosis is used to describe a systemic disease characterized by skin induration and thickening accompanied by various degrees of tissue fibrosis and chronic inflammatory infiltration in numerous visceral organs, prominent fibroproliferative vasculopathy, and humoral and cellular immune alterations.
The American College of Rheumatology (ACR) criteria for the classification of systemic sclerosis require one major criterion or two minor criteria, as follows:

• Major criterion: Proximal scleroderma is characterized by symmetric thickening, tightening, and induration of the skin of the fingers and the skin that is proximal to the metacarpophalangeal or metatarsophalangeal joints. These changes may affect the entire extremity, face, neck, and trunk (thorax and abdomen; see images below). Tightening of the skin in the face, with a characteristic beaklike facies and paucity of wrinkles. Sclerodactyly with digital ulceration, loss of skin creases, joint contractures, and sparse hair.
• Minor criteria

  • Sclerodactyly is characterized by thickening, induration, and tightening of the skin, limited to only the fingers.
  • Digital pitting scars or a loss of substance from the finger pad: As a result of ischemia, depressed areas of the fingertips or a loss of digital pad tissue occurs.
  • Bibasilar pulmonary fibrosis includes a bilateral reticular pattern of linear or lineonodular densities most pronounced in basilar portions of the lungs on standard chest roentgenography. These densities may assume the appearance of diffuse mottling or a honeycomb lung and are not attributable to primary lung disea