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Dermatopathology: Practical & Conceptual October - December 2001
Hypothesis: Degos’ Disease Is a Distinctive Pattern, Chiefly of Lupus Erythematosus, And Not a Specific Disease
Elízabeth Ball, M.D.
Amy Newburger, M.D.
A. Bernard Ackerman, M.D.
The Patient Who Sparked Our Interest
Histopathologic Findings In Chronologic Sequence
Course and Prognosis
Cause and Mechanism
The Association of Degos’ Disease and “Collagen Vascular Disorders”
Our Concept of Degos’ Disease
Although the disease under consideration here bears the name "Degos," it actually was described first in 1941 by Köhlmeier, an Austrian. He observed peculiar atrophic skin lesions in a 22-year-old man who died as a complication of ulcers of the intestine. The process was thought by Köhlmeier to be "thromboangiitis obliterans of the intestine with multiple skin lesions" (
Figs. 3A and 3B
In 1942, Degos, Delort, and Tricot reported on a 49-year-old plumber with a condition they initially called "dermatite papulo-squameuse atrophiante." The patient sought consultation for an eruption that was characterized by Degos (
pale rose papules, most numerous on the trunk and measuring 23 mm diameter. Within 2 or 3 days they became umbilicated and flattened out to form irregular patches, sometimes linear, whose size never exceeded 10 x 3 mm. The central area of these patches was white, depressed, atrophic and covered by a squamous non-adherent crust; surrounding this atrophic zone was a reddened area congested or finely telangiectatic, fading to a simple filiform border in the oldest lesions. The eruption progressed in small bouts of 3 or 4 elements each week, without the complete disappeareance meanwhile of the older lesions. The mucosae were not affected."
Figs. 3A, B Skin lesions in the patient described as "thrombangiitis obliterans" by Köhlmeier in 1941.
Fig. 4 Professor Robert Degos circa 1942.
Those coauthors regarded the lesions as distinctive and, unaware of the publication of Köhlmeier, opined that they had not been described previously. In May 1942, seven months after the onset of his illness, the patient died after having developed symptoms and signs of an acute gastrointestinal process. At post mortem examination, yellow lesions in the small intestine were found to house thrombi in venules as well as an infiltrate of polymorphonuclear leukocytes. The autopsy report commented thus: "
The lesions in the small intestine, macroscopically and microscopically, were like those in the skin."
Because of the fatal outcome, Degos
in 1948, changed the name of the condition to "papulose atrophiante maligne."
also in 1948, recorded findings in another patient with the same malady and compared the lesions in the skin to drops of porcelain with an erythematous rim, dubbing them "
Ulery-theme porcelain en gouttes."
The first patient with malignant atrophic papulosis recorded in the United States was by Nay-lor
Nomland and Leyton, in 1960 too, called attention to involvement by the disorder of the central nervous system and told of vascular lesions in the heart and the kidneys.
Subsequently, the condition was written about by Gever,
In 1974, Degos and Kalis reviewed the world's medical literature about malignant atrophic papulosis. By that time, 63 patients were thought by them to have had the disease, and of those 38 patients died less than two years after the appearance of skin lesions. Two patients survived for more than 15 years.
By 1998, about 130 patients had been reported on.
in 1989, reviewed the records of 111 patients about whom information sufficient to establish the diagnosis was available. This is what they found:
Male to female ratio: 1.3: 1. Males may be affected more severely than females.
Age at diagnosis: 3 weeks to 67 years; few infants and few patients over 60 years of age.
Survival: 54 of 111 patients died and of these 32 died within two years of diagnosis. The most common cause of death was peritonitis (62 % of fatalities).
During the mid 1980's, reports began to appear of patients with Degos' disease having lesions limited to the skin.
Many of those patients had relatives who were affected in similar fashion, that is, with typical skin lesions but no visceral manifestations.
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