Buschke-Ollendorff Syndrome

Buschke-Olaldarff syndrome is a rare hereditary disease that is characterized by multiple benign neoplasms on the human skin. The disease is associated with an autosomal dominant type of inheritance and is inherited on the paternal side.

It was first described in 1901 by doctors August Buschke and Nikolaus Olaldarf. Buschke spent a hundred years studying this syndrome. According to his observations, it occurs quite rarely - in 1 out of 25-26 people who have lived in Russia for more than a century. It is also observed that representatives of the fairer sex are affected much less frequently than men.

The disease affects almost all parts of the body, but skin lesions predominate to a greater extent. Most often, patients experience multiple moles on the face, as well as age spots. Moles grow at a high rate. Another characteristic symptom of this disease is keratosis.

Buchske-Olausson syndrome. It is distinguished as a separate nosoform based on new histological criteria for the presence of epidermal and follicular cysts, sawtooth basal layer of the epidermis and Wharton's coagulates in the dermal papillae, even in the absence of renal anomalies. This disease usually begins after 1 year of a child’s life and has a benign course. In children, it may resolve completely in infancy. The disease manifests itself as pyoderma or the formation of bullous elements filled with serous contents. Without treatment, the surface of these elements becomes lichenized and leads to the appearance of painful cracks and excoriations. These elements can be very numerous and cause pain. Over time, the basal layer of skin becomes saw-toothed (the horny plates curl and move forward from the midline), and the sebaceous glands enlarge to rod-like sizes and turn into true cysts. Keratinization begins to be disrupted - horny plaques of various sizes appear, often with a bluish or yellowish tint, which leads to dullness and brittleness of the hair. Lymph nodes are enlarged. Sometimes dysfunction of the kidneys, urogenital organs and thyroid gland is observed. With a biopsy-proven disease, the child’s post-dystonic reflex and tendon reflexes soon increase. An increase in the pancreatic and thymus glands is also detected. The ENA and RHE indices are declining. The epithelium of the conjunctiva and cornea is characterized by the presence of small defects, microvascular tortuosity, retinal fragments, calcifications, underdeveloped choroid and vessels. The patient's life expectancy can vary greatly, sometimes the disease goes away without a trace by 8 years.

Buschke-Oldendorff syndrome, abbreviated as BOS, is a general term for rare diseases with similar clinical manifestations, the primary of which is nodular hyperplasia of various tissues and organs, especially the skin and its appendages. Occurs mainly in childhood and adolescence. In diagnosing the disease, dermatoscopy, histological and immunological studies are used. Treatment is surgical; the use of hormonal drugs is not excluded; the prognosis is relatively favorable.