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Juvenile polyfibromatosis of Rhine fingers: causes, symptoms, diagnosis, treatment
Last reviewed: 20.11.2021
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The causes and pathogenesis of juvenile polyfibromatosis of the Rhine's fingers have not been fully established. It is believed that dermatosis has an autosomal dominant type of inheritance.
Symptoms of juvenile polyfibromatosis of the fingers of the Rhine. Characteristic development of dermatosis since birth or in the first months of life with the formation of fibrous nodular growths in the interphalangeal regions of the fingers and feet. Fibrous formations (tumors) of round, rarely hemispherical, pinkish, flesh-colored or normal skin color, single or multiple, of various sizes. Tumors have a shiny surface, a dense consistency and palpation is painless. Juvenile polyfibromatosis of the Rhine's fingers can lead to deformations and contractures of the fingers and toes. The cases of combination of juvenile polyfibromatosis of fingers with other hereditary dermatoses are described.
Histopathology. In the dermis and subcutaneous tissue there are nodular formations, consisting of cells of the fibroplastic series and a large number of collagen fibers. In the cytoplasm of myofibroblasts, single or multiple round forms of small inclusions are determined.
Treatment of juvenile polyfibromatosis of the fingers of the Rhine. Recommended surgical excision, corticosteroid ointment for occlusive dressing. With the progression of the process, oral neotigazone is effective.