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INTRODUCTION
Morphea is a clinically distinct inflammatory disease, primarily of the dermis and subcutaneous fat, which ultimately leads to a scar-like sclerosis. The small vessel changes, inflammatory infiltrate, and ultimate structural modifications are identical in morphea and systemic sclerosis (SSc), but the two diseases are distinct entities that can be distinguished clinically. In addition, their triggers presumably differ and coexistence of morphea and SSc is rare. Morphea has an asymmetric patchy or linear distribution. In contrast, SSc begins either as symmetric tightening of the fingers and hands that extends progressively toward the forearms (limited cutaneous SSc [lcSSc]) or as symmetrically extending sclerosis of the trunk and proximal upper extremities (diffuse cutaneous SSc [dcSSc]). Rarely, generalized morphea may resemble early dcSSc. The presence of Raynaud phenomenon, digital sclerosis, and involvement of the gastrointestinal tract and the lung also allows differentiation of SSc from morphea. Distinguishing between deep morphea and eosinophilic fasciitis can prove more difficult โ whether these two disorders represent distinct entities or simply the ends of a broad clinical spectrum remains debatable, e.g. eosinophils may be present at the inflammatory border of morphea.
Morphea can cause significant morbidity as a result of pain, skin tightness, and impaired joint mobility. In ~10% of patients with morphea, sclerosis leads to significant contractures, growth retardation, and even micromelia, resulting in long-term functional disability. Disease activity correlates with reduced quality of life.