Linear scleroderma with Parry–Romberg syndrome. A clinical case

Introduction. Scleroderma is a chronic skin disease based on connective tissue damage. There are two forms of scleroderma: systemic (with internal organ damage, treated by rheumatologists and therapists) and chronic cutaneous (without internal organ damage, treated by dermatologists). The clinical manifestations of skin forms of scleroderma are age spots, skin induration and atrophy. The term «scleroderma» or «hard skin» was introduced into science in 1643, after which there was a rather long period (more than three centuries), called «dermatological», when only skinmanifestations of scleroderma were studied. Only in the middle of the XX century, the concept of a possible systemic nature of scleroderma (Clemperer’s concept of «collagen diseases») appeared, and intensive study of visceral pathology in scleroderma began. Purpose – was to present a case of linear saber scleroderma in combination with Parry–Romberg syndrome. Materials and methods. We present a clinical case of patient B, 25 years old, who was diagnosed with linear (saber-shaped) scleroderma with Parry–Romberg syndrome: atrophy of the muscles of the right side of the face, lesions with atrophy of bone structures (atrophy of the upper jaw, teeth), lesions of the nervous system (trigeminal neuropathy, Jackson’s epilepsy pyramidal insufficiency) in combination with systemic sclerosis with vascular lesions (Raynaud’s syndrome), heart (myocarditis without heart failure, active stage with foci of fibrosis), peripheral sensory demyelinating polyneuropathy. Results. The article presents clinical features of the disease, typical changes detected by MRI and CT of the abdominal cavity, typical laboratory characteristics (use of modern laboratory biomarkers of the disease), which provide the greatest opportunities for diagnosing this disease and possible methods of treatment. Conclusions. We report a rare case of linear (saber-shaped) scleroderma with Parry–Romberg syndrome, diagnostic methods, and treatment options.