Primary atrophic profound linear scleroderma - Report of three cases

We present 3 unusual cases of deep linear, primary atrophic scleroderma, no t preceded by inflammatory reaction and sclerosis, involving the subcutis a nd deeper tissues. These cases differ in the course and prognosis from typi cal profound scleroderma since they do not lead to disfiguration and crippl ing deformities. In contrast to the atrophies left after regression of morp hea or linear scleroderma, they do not involve the dermis, which does not s how discoloration or changes in texture. Infiltrates in the endomysium, inv olvement of deeper tissues and the progressive character of the disease arg ue for atypical primary atrophic profound scleroderma. The coexistence in o ne case of primary facial hemiatrophy appears to indicate also its relation ship with primary linear atrophies of the limbs. Copyright (R) 2000 by S. K arger AG, Basel.