Generalized or diffuse scleroderma is a disease well studied from the clinical and the microscopic point of view and certainly is a well defined clinical entity. It is not restricted to the skin and to the organs adjacent to the skin but is a generalized systemic disease of the connective tissue.
In most cases the process starts on the hands and feet with a somewhat later and slower involvement of the face.
Three stages can be distinguished clinically: the edematous, the indurative and the atrophic stage. The initial edema does not pit on pressure. The skin appears tense and cannot be folded. The face assumes a masklike expression; the regular folds smooth out. In the second phase the skin hardens and stiffens. This is particularly pronounced on the fingers, on the dorsa of the hands and in the region of the ankles. Hyperpigmented and depigmented spots appear in this stage.
LINDSAY JR, TEMPLETON FE, ROTHMAN S. LESIONS OF THE ESOPHAGUS IN GENERALIZED PROGRESSIVE SCLERODERMA. JAMA. 1943;123(12):745–750. doi:10.1001/jama.1943.02840470011003
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