From Big Medical Encyclopedia

YaDASSONA-LEVANDOVSKOGO the SYNDROME (J. Jadassohn, 1863 — 1936, is mute. dermatologist; F. Lewandow-sky, 1879 — 1921, is mute. dermatologist; synonym: the pachyonychia is inborn, a pachyonychia ikhtioziformny, a polydisplasia ectodermal inborn giperklastichesky type of Touraine — Gisselbrekhta, a polykeratosis kongenitalny, Siemens a mnogoformny keratosis, Schaefer's syndrome) — the hereditary monogenic defect which is shown damage of nails, a focal keratosis of skin, a leukokeratosis of mucous membranes. The disease, apparently, is inherited on autosomal dominantly type.

At this disease in epidermis the hyperkeratosis (see) with a focal parakeratosis (see), an acanthosis (see), places vacuolation of cells, destruction of intercellular bonds, a dyskeratosis is noted (see); in a derma — small perivascular infiltrates; in a nail matrix and hypo-nikhii — a hyperkeratosis and a dyskeratosis.

The wedge, symptoms appear since the birth or in the first 3 — 5 years of life. The basic and the most precursory symptom is the pachyonychia on fingers of hands and legs; at the same time nail plates are thickened, dense, muddy, whitish or yellowy-brown color, a cylindrical form with considerable camber, narrowing to free edge of a nail, rising in a distal part that is caused by a hyponychial keratosis. Recurrent inflammatory paronychias can be observed (see) and rejection of nails.

Sites of a keratosis (the strengthened keratinization) develop on skin of soles, especially in places of pressure, and on palms, and also on finger-tips, a nose, on anklebones, buttocks. Focal giperkeratoza are symmetrized, are accurately delimited, on soles quite often are followed by formation of painful bubbles. Insular borodavchatopodobny keratoza appear on a lap, elbows, shins, the back of joints of fingers, in subnodal folds. Reddish Akneformny follicular keratotichesky papules arise on a nose, in a mental fold, on a waist, buttocks, an inner surface of hips, in large folds and on other sites of skin. The peeling of skin of a body, a pilar part of the head is sometimes noted. The hyperhidrosis is characteristic (see) palms and soles.

The centers of a leukokeratosis are found hl. obr. on a mucous membrane of an oral cavity, an anal orifice. At nek-ry patients keratotichesky changes of tympanic membranes, defeat of a cornea of eyes, the lacrimal tubules are observed. The malignancy of the centers of a leukokeratosis, infection of skin in the field of bubbles and nail rollers, decrease in sight are possible.

The diagnosis is established on the basis by a wedge, pictures. Differential diagnosis carry out with keratodermias (see Keratoza), various defects included in group of polykeratoses of Touraine (see Tu turnip a polykeratosis), the acquired and hereditary damages of nails (see Nails).

Hyponychial giperkeratotichesky masses deletes with a scalpel. Apply soap and soda bathtubs, keratolytic drugs in mix with Dimexidum to a softening of keratoz of skin and nails. At emergence of bubbles and paronychias use fukortsin, 1% lincomycin paste, 2% resorcinol paste and other antibacterial agents. Use of the fortifying and improving microcirculation means is reasonable (vitamins, biogenic stimulators, etc.).

The course of a disease chronic, the malignancy of the centers of a leukokeratosis is possible.

Dispensary observation for patients is necessary. For reduction of formation of bottom keratoz special footwear with soft laying is recommended. Bibliography: Ariyevich A. M. and She-

of a tsirula of JI. T. Patologiya of nails, Tbilisi, 1976; The Dermatological sindromo-logiya, under the editorship of R. S. Babayants, Yerevan, 1974; Differential diagnosis of skin diseases, under the editorship of A. A. Studnitsin, M., 1983; Suvorova K. H., P about y M. F. and Levitin N. I. Sindr Yadassona's t-burd — Lewandowski,

Vestn. dermas, and veins., No. 8, page 62, 1974; Jadassohn J. u. Lewandow-s k at F. Pachyonychia congenita, in kN: Jakobis Ikonogr. derm., B., 1906; M about 1-denhauer E. u. Ernst E. Das Jadassohn — Lewandowsky-Syndrom, Haut-arzt, Bd 19, S. 441, 1968; Voglino A. o. Pachionichia congenita, Chron. Derm., p. 625, 1982.

A. A. Antonyev, To. H. Suvorova.