DERMATOFIBROZ LENTIKULYARNY DISSEMINATED

From Big Medical Encyclopedia

DERMATOFIBROZ LENTIKULYARNY DISSEMINATED (dermatofibrosis lenticularis disseminata; Greek derma, dermat[os] skin + lat. fibra fiber + - osis; lat. lenticularis lenticular; lat. disseminatus scattered; synonym Bushke's syndrome — Ollendorff) — the rare genodermatosis which is characterized by multiple small deratofibromas.

A. Bushke and Ollendorff (N. of Ollendorff) in 1928 is described for the first time. Modern scientists consider D. of l. as manifestation of a polyfibromatosis of Touraine (see the Deratofibroma); it is inherited it is prepotent, women are ill more often. Cases of development of a syndrome in newborns are known. At sick D.' half of l. is combined with an osteopathy of long tubular bones, sometimes with frustration of mentality, diabetes, obesity, disturbance of an ovarian cycle, a disease of Recklinghausen, hair it is deprived.

Multiple deratofibromas in a waist at the lentikulyarny disseminated dermatofibroz (at the left above — character of an arrangement of rash).

Patogistologiya: not really accurately delimited dermogipodermalny fibromas, usually without elastic fibers; under the lower bound of epidermis the strip of normal connecting fabric remains.

A clinical picture

Rashes represent the lenticular, roundish or oval hemispherical a little flattened dense educations, to dia, from 0,3 to 1,5 cm, with sharp borders, colors of normal skin, yellowish or brownish-red; on slightly brilliant surface of epidermis there can be an insignificant peeling, teleangiectasias, small hemorrhages. Rashes arise gradually and are located absent-mindedly or densely, is usually symmetric in the field of a shoulder girdle, a waist (fig.), a stomach, buttocks, on a flexion surface of distal department of extremities. In several years on places of some rashes there is a pigmented cicatricial atrophy of type anetodermas (see); at the same time there are new rashes.

The diagnosis

the Diagnosis does not cause difficulties; differentiate with pseudoxanthoma elastic (see), hyalinosis (see), leiomyoma (see). Cooling or friction of rashes of leiomyomas causes pain, swelling and their reddening. Rash at a hyalinosis (lipoid) usually of a polimorfn with bubbles and pustules, affects face skin, mucous membranes of a mouth and a throat (an osiply voice) more often. The centers of a pseudoxanthoma elastic remind so-called pebble-leather with teleangiectasias, have a mesh appearance, are well delimited, are located on a neck, in large folds, are combined with damage of eyes and arterial system.

Treatment

Treatment is a little effective; operational removal of a large number of deratofibromas is impossible, diathermocoagulation quite often comes to the end with formation of hypertrophic hems.

Forecast for life favorable.

See also Genodermatoses , Teleangiectasia .


Bibliography: Dermatological sindromologiya, under the editorship of R. S. Babayants, page 170, Yerevan, 1974; Buschke A. u. Ollendorff H. Ein Fall von Dermatofibrosis lenticularis disseminata und Osteopathia condensans disseminata, Derm. Wschr., Bd 86, S. 257, 1928; GrupperC h. et Cardinne A. Dermatofibrose len-ticulaire disseminee avec osteopoecilie (pere et fils), Syndrome de Buschke — Ollendorff, Ann. Derm. Syph. (Paris), t. 101, p. 405, 1974, bibliogr.

H. A. Torsuyev.

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