Sclerosing disorders of the skin consist of a heterogeneous spectrum of entities that share in common cutaneous sclerosis with excessive local accumulation of collagen and/or other extracellular matrix components in the dermis, subcutaneous tissue, and/or underlying soft tissues. The clinical course of these diseases varies from benign disease with a localized skin involvement to systemic, life-threatening disorders. Thus, a correct diagnosis is extremely significant as these disorders are very different in terms of pathogenesis, course, treatment, and outcome. Although they have sclerosis as an overlapping feature, other histopathological features, such as sclerosis depth, hyalinization, mucin deposition, sclerotic bodies, and fibroblastic proliferation, may provide clues to a more specific diagnosis. In doubtful cases, clinicopathologic correlation, immunohistochemical staining, and other laboratory data may be required to arrive at a proper diagnosis.
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