Abstract
Morphea is a rare sclerotic autoimmune disorder primary affecting the skin and subcutaneous tissues. The linear head variants involve the facial area, with asymmetries and deformities. Eighteen patients with hemifacial deformity (age range 14–75 years) were assessed before surgery (T0), and after one (T1, 18 patients) or two (T2, six patients) surgical treatments of facial autologous fat grafting. A stereophotogrammetric reconstruction of the facial surface was obtained for each patient and a group of control subjects, and facial symmetry was quantified according to the root mean square distance between homologous areas of trigeminal innervation. Values obtained from the control subjects were used to calculate z-scores for patients. At T0, all facial thirds of the patients resulted significantly more asymmetrical than those of the control subjects (Mann–Whitney test, p < 0.05), while at T1, the symmetry of the middle facial third did not differ from that of control subjects (p = 0.263). At T2, the upper and the lower facial thirds also did not differ from the control values (p > 0.05). The faster result obtained in the facial middle third was in accord with clinical findings. In conclusion, autologous fat grafting significantly improved facial asymmetry after one (middle facial third) or two (lower and upper thirds) treatments; the outcomes were efficaciously quantified by stereophotogrammetry.
Highlights
Morphea is a rare, clinically heterogeneous, sclerotic autoimmune disorder characterized by increased and excessive collagen deposition, whose pathogenesis, not fully understood, is likely multifactorial
Among the 18 patients (11 females and 7 males) with a head-variant linear morphea condition, seven patients were diagnosed with En Coupe de Sabre scleroderma syndrome (ECDS), seven with Parry–Romberg syndrome (PRS) and four generically with linear scleroderma, involving the face, unilaterally
All patients presented unilateral involvements: upper third unilateral lesions were diagnosed in 14 patients, middle third lesions in 13 patients while the lower third resulted the less involved and only nine patients were diagnosed with skin scleroderma lesions, relating to this facial third
Summary
Clinically heterogeneous, sclerotic autoimmune disorder characterized by increased and excessive collagen deposition, whose pathogenesis, not fully understood, is likely multifactorial. It is recognized as localized scleroderma and includes disorders characterized by sclerosis primary affecting the skin, but it can involve adjacent subcutaneous tissues in either a limited or extensive fashion. It begins with an initial inflammatory phase followed by subsequent sclerotic and/or atrophic phases: damage to vessels, profibrotic cytokine release, and the unbalance of collagen production and destruction represent all the culminating disease effects [1]. Other classification systems were proposed and more widely adopted in clinical settings
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