DEGOSA SYNDROME

From Big Medical Encyclopedia

DEGOSA SYNDROME ( R. Degos , fr. dermatologist-syphilologist, sort. in 1904; synonym: papulosis atrophicans maligna, Degos's syndrome — Delora — Body stockings) — seldom found symptom complex of not clear etiology which is characterized by damage of skin also went. - kish. path. It is for the first time described by W. Kohlmeier in 1940; it is in detail studied by R. Degos, Delor and Body stockings (J. Delort, R. Tricot) in 1942, 1948. Most of modern writers carries D. of page to vasculites — system necrotizing angiites.

the Greatest changes are noted by Patogistologiya in vessels of a derma — trombarteriolit with the subsequent heart attack; walls of some vessels and collagenic fibers of a nekrotizirovana around them; in walls of vessels adjournment of immunoglobulins; in perivascular zones a hyaline degeneration of collagenic fibers, sometimes infiltrate from lymphocytes and histiocytes. Similar changes can arise in larger vessels of a derma and a hypodermic fatty tissue, and also in vessels of a wall of intestines and stomach.

The clinical picture

the Disease is observed at men. Initial sign is the enanthesis of papules of light pink color, a hemispherical form, to dia. 3 — 4 mm, quite often edematous, reminding a blister. Favourite localization — a trunk, a nape, is more rare than an extremity. In several days the center of a papule becomes covered by a scale, after removal the cut is noted the superficial atrophic impression of phosphorus-white color which is gradually taking almost all surface of an element, except the narrow slightly towering regional rim; on it slightly expanded capillaries clearly are visible. After several weeks of a papule disappear, leaving resistant ospennovidny atrophic scars. After skin rashes in several weeks, months, and sometimes and years defeats develop went. - kish. a path (multiple perforation, preferential small intestine) which first sign are sharp pristupoobrazny abdominal pains, more intensively on the right, symptoms of «acute abdomen» develop. As a rule, the attack arises in several hours after food and comes to an end with a plentiful diarrhea.

Character and localization of pains can sometimes simulate an ulcer of a duodenum.

At D. of page defeat of a nervous system (a head and spinal cord, peripheral nerves), an eye is possible (rashes on a sclera, changes of an eyeground — tortuosity and irregularity of veins and dot yellow ochazhka). Isolated cases of damage of visible mucous membranes are described (oral cavities, generative organs). Morphology of blood usually without aberrations, a proteinemia, a bilirubinemia.

Disease is usually rough, heavy, occasionally more favorable.

Diagnosis it is put on the basis of the typical skin rashes which are followed went. - kish. frustration, in doubtful cases — as a result gistol, researches of skin.

Treatment

Corticosteroids, anticoagulants, indometacin, antibiotics of a broad spectrum of activity, vitamins, hemotransfusions, gamma-globulin. In cases of development of symptoms of an acute abdomen an operative measure.

Forecast

Always serious; quite often painful attack accompanies perforative peritonitis, a collapse with a lethal outcome; death is caused and exhaustions in connection with defeat went. - kish. path. Patients seldom live 3 — 6 months after emergence of the first signs of damage of intestines.


Bibliography: Arutyunov V. Ya. and P. I Golemba. Allergic vasculites of skin, M., 1966, bibliogr.; Shaposhnikov O. K. and Demenkova N. V. Vascular damages of skin, L., 1974, bibliogr.; Degos R., Delort I. et Tricot R. Dermatite papulo-squameuse atrophiante, Bull. Soc. fran<?. Derm. Syph., t. 49, p. 148, 1942; To 6 h 1 m e i e r W. Multiple Hautnekrosen bei Thrombangiitis obliterans, Arch. Derm. Syph. (Berl.), Bd 181, S. 783, 1941; N i with o 1 an u S. With &h. si BadnoiuAl. Elemente de dermatologie fiziopatologica, Bucure§ti, 1967.

G. B. Belenky.

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