Juvenile polyfibromatosis of Rayne's fingers: causes, symptoms, diagnosis, treatment

The causes and pathogenesis of juvenile polyfibromatosis of the fingers of Rayne have not been fully established. It is believed that the dermatosis has an autosomal dominant type of inheritance.

Symptoms of juvenile polyfibromatosis of the fingers of Rayne. The development of the dermatosis is characteristic from birth or in the first months of life with the formation of fibrous nodular growths in the interphalangeal areas of the fingers and toes. Fibrous formations (tumors) are round, rarely hemispherical, pinkish, flesh-colored or the color of normal skin, single or multiple, of various sizes. The tumors have a shiny surface, dense consistency and are painless upon palpation. Juvenile polyfibromatosis of the fingers of Rayne can lead to deformations and contractures of the fingers of the hands and toes. Cases of a combination of juvenile polyfibromatosis of the fingers with other hereditary dermatoses have been described.

Histopathology. In the dermis and subcutaneous tissue there are nodular formations consisting of fibroplastic cells and a large number of collagen fibers. In the cytoplasm of myofibroblasts, single or multiple small round inclusions are determined.

Treatment of juvenile polyfibromatosis of the fingers of Rayne. Surgical excision, corticosteroid ointments under an occlusive dressing are recommended. With the progression of the process, the oral administration of neotigazone is effective.