DEVERZHI DISEASE

From Big Medical Encyclopedia

DEVERZHI DISEASE (M of G. And. Devergie, fr. doctor, 1798 — 1879; synonym: red sharp-pointed deprive of Gebra — Kaposha, red scaly hair deprive, pityriasis rubra pilaris) — a chronic dermatosis, primary element to-rogo is a follicular papule with a scaly peeling. The disease is described in 1835 Mr. of P. Rayer under the name the general psoriasis. In 1856 Mr. of Deverzhi in detail described and allocated a disease in an independent nozol, a form, having offered the name pityriasis pilaris. Some modern writers consider Must be as one of forms keratoz (see).

The etiology

the Aetiology is unknown; in the past the opinion on a tubercular origin prevailed Must be, and also about communication of this disease with a toxoplasmosis. There are observations testimonial of an undoubted role of neuroendocrinal frustration, and also that in certain cases the dermatosis develops soon after an acute infectious disease. Family diseases are known (transfer on the dominant line) from the beginning of manifestation at children's age, however in most cases it is not possible to track a role of hereditary factors. In 1941 Brunsting and Shird (L. Brunsting, S. of Sheard) put forward the theory of deficit of vitamin A as the reason of development there is

Patogistologiya

the Follicular hyperkeratosis, places a diffusion, focal parakeratosis, uneven granulez, an acanthosis, vacuolation of cells of a basal layer; in a papillary layer of a derma moderate inflammatory infiltrate preferential from segmentoyaderny leukocytes and lymphocytes, hl. obr. around vessels; grease and sweat glands often atrofichna.

Clinical picture

Fig. 7 and 8. Rashes on a brush and a knee joint at Deverzhi's disease (the follicular conic papules which are located close from each other).
Fig. 9. Elements of rash at increase.

The disease begins at any age, develops gradually, more rare sharply, proceeds chronically. Allocate the generalized, localized and abortal forms.

A generalized form — the most typical display of a disease. Primary element of rash is the follicular conic papule of the wrong outline, to 1,5 mm in the diameter, brown-red or pink color, covered at top with scaly scales or a horn spinule. Papules are located close from each other why the center of defeat to the touch reminds a surface of a grater. Scales densely cover one or several vellus hair which are crooked or atrofichna. The quantity of papules increases over time, they merge in reddish extensive plaques. Plaques considerably infiltrirovana, a surface their dry, is shelled by small scaly or lamellar scales. Rashes are usually symmetric and have favourite localization: a pilar part of the head, face skin in the field of nasolabial folds, a dorsum of fingers of hands, elbow and knee joints (tsvetn. fig. 7 — 9), an outer surface of forearms and hips, palms and soles. On elbows and knees of a plaque usually bright red, reminding psoriasis (with the yellow, firm, densely sitting scales). On skin of a pilar part of the head the scaly peeling is observed. Face skin is as if tense, sharply hyperemic, is shelled by small scales. On a dorsum of fingers of hands, as a rule, there are red shelled plaques or blackish horn traffic jams in mouths of hair follicles (so-called cones of Benye) that is considered a pathognomonic sign Must be. Nail plates often grow dull, gain burozhelty color and longitudinal striation, highly rise over a bed at the expense of a hyponychial hyperkeratosis. On palms and soles skin becomes brown-red, the corneous layer is sharply thickened, cracks are formed. Subjective feelings are almost absent; sometimes the feeling of tightening of skin, burning, an itch and a chill disturbs. Even at considerable distribution of process there are small intervals of healthy skin which have angular contours and bear on themselves separate horn thorns. Seldom or never the conjunctiva a century, a mucous membrane of a mouth is involved in process, on a hard palate there are small sharp-pointed small knots of pink color. Arthritis is even more seldom observed. In the past cases of a heavy current were described Must be with a cachexia and even a lethal outcome.

At the localized form of a disease small sites of skin are surprised, fingers, pilar head skin, forearms or palms and soles are more often.

The abortal form differs in the fact that expressiveness of an erythema, papular rashes, infiltrate and a peeling is sharply reduced.

The diagnosis is based on a wedge, data (especially existence of cones of Benye on a dorsum of fingers of hands) and gistol, a picture (a follicular hyperkeratosis, etc.).

Treatment

Prolonged use of vitamin A in high doses (at the same time it is necessary to consider a condition of a liver) in combination with ascorbic to - that and vitamins of group B; repeated courses of treatment are appointed at an interval of 2 — 3 months; are sometimes effective hydrazide isonicotinic to - you, Chingaminum (Resochinum, delagil), etc. At identification of endocrine disturbances treatment is shown by the corresponding pharmaceuticals. In hard cases of a generalized form Must be appoint corticosteroids inside, a methotrexate. Heat baths, 1 — 2% salicylic and sulfur-salicylic ointments, in a phase of regress uv radiations are shown.

The forecast

the Forecast concerning life favorable: there can be remissions; spontaneous treatment is extremely seldom observed. Treatment does not warn a recurrence more often.


Bibliography: The multivolume guide to a dermatovenereology, under the editorship of S. T. Pavlov, t. 3, page 415, M., 1964; Dever-g i e M. G. A. Pityriasis pilaris, mala die de peau non decrite par les derma tologistes, Gaz. hebd. de of m£d. Paris, t. 3, p. 197, 1856; Gertler W. Sys-tematische Dermatologie und Grenzge-biete, Bd 2, S. 830, Lpz., 1972.

Yu. Ya. Ashmarin.

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