Clinical appearance of skin lesions and disturbances of pigmentation in localized scleroderma

Skin manifestations of localized scleroderma were assessed clinically in 58 patients presenting 214 circumscribed scleroderma lesions (23 patients with localized morphoea plaques, 15 with generalized morphoea, 11 with linear scleroderma of trunk and extremities, 9 with scleroderma en coup de sabre). Characteristic differences between different types of localized scleroderma with respect to age at debut, regional distribution, symmetry and linearity, degree of sclerosis of the lesions, and the presence of inflammatory change of colour, pigmentation and visible atrophy of underlying subcutaneous tissue were found. Separate brown-pigmented spots resembling atrophoderma. Pasini-Pierini were observed in 49% of the patients. A regional distribution chart of linearity in localized scleroderma was elaborated. The frequent finding of disturbances of pigmentation, the character of the linear distribution-pattern, and affections of underlying anatomical structures is discussed to indicate a predisposing defect in the migration of crest cells during embryonal life.