Scleroderma: Difficulties in classification, diagnosis, treatment

Abstract

Localized scleroderma (LoS) is an autoimmune connective tissue disease with a variety of clinical manifestations. The dermatologist faces the difficulties of establishing a diagnosis and choosing the most appropriate therapy due to the relatively rare prevalence. This review article explores the challenges of classification, diagnosis, and treatment, as well as the factors that contribute to their occurrence. The greatest difficulties in diagnosing LoS that affected the prognosis and progression of the illness are: a delay in diagnosis, including early detection; omission of the active stage of LoS; difficulties in differential diagnosis; lack of association with Lyme borreliosis; low diagnostic value of laboratory and histological tests; limited use of sensitive instrumental methods for diagnosis; and monitoring the activity of LoS in a doctor’s practice. The main difficulties in treatment are the delay in starting treatment; the use of therapy methods with low evidence-based effectiveness; the rare prescription of highly effective approaches; the insufficient effectiveness of systemic drugs and phototherapy; difficulties in determining the volume, timing, and regimen of therapy for each subtype of LoS; the difficulty of achieving a remission; dependence of treatment approach on the doctor’s specialization; the lack of targeted drugs with evidence-based effectiveness in LoS; the lack of high-level evidence-based effectiveness and safety of therapy in LoS; difficulties in the correction of complications of Lo S.