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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
Degos' disease usually presents itself in the skin in the form of asymptomatic lesions that range in number from few to hundreds and that are situated predominantly on the trunk and upper limbs. The palms, soles, face, scalp, and genitalia tend to be spared, although there are exceptions.
Early the typical lesion is a pink macule that soon becomes a small (between 25 mm in diameter), red, round, smooth, firm papule. Within a few weeks, the papule becomes umbilicated. An individual lesion typically consists of two zones, a depressed porcelain white scaly center and a rose or violaceous rim, 12 mm wide, made up in part of delicate telangiectasias. In time, the reddish border disappears and only a varicelliform white scar remains. A fully formed lesion usually is less than 1 cm in diameter and rarely exceeds 2 cm.
The mucous membrane involved most often is ocular and in the eye the most common manifestation is an avascular patch on conjunctivae; sclerae, episclerae, retinae, choroids, and optic nerves may be affected.
An adverse prognosis is telegraphed by involvement of the gastrointestinal tract that, only episodically, precedes manifestations in the skin.
After weeks, months, or even years of the appearance of new skin lesions, a patient may have episodes of abdominal pain, nausea, melena, and diarrhea. The constellation of symptoms and signs may simulate those of pancreatitis, gastritis, peptic ulceration, ileus, and dysentery.
Laparo-scopy reveals lesions in different parts of the small intestine that are very similar in appearance to those in the skin. The lesions are infarcts; those of similar character have been noted in the stomach, colon, duodenum, esophagus, and rectum.
Involvement of the bowel may lead to perforation, peritonitis, and death.
Neurological signs and symptoms occur in 22% of patients with Degos' disease,
mostly as a result of infarcts in the brain and spinal cord.
The largest series of patients is that of Subbiah
who studied, prospectively, medical records of 15 patients. The mean duration of follow up was 13 years. The initial manifestation in 13 patients was in the skin, in one patient the gastrointestinal tract, and in one patient the central nervous system. The duration of signs and symptoms prior to diagnosis ranged from one to 16 years, with a mean of two years. Ten patients developed neurologic manifestations
that vary according to site of involvement as follows: hemiparesis, aphasia, monoplegias, flaccid quadriplegia, sensory abnormalities, ptosis, optic neuritis leading to blindness, diplopia, diminution of visual fields, and papilledema. Death consequent to involvement of the brain is exceptional, but dementia and meningo-encephalitis presage eventual coma and an untoward end.
At autopsy there are multiple, small, hemorrhagic infarcts grossly and thrombo-sis of arteries with extensive intravascular and ex-tra-vascular deposits of fibrin histo-patho-logically.
At post-mortem examination, infarcts have been found in the myocardium, pericardium, kidneys, bladder, lungs, pleura, liver, and pancreas.
They usually are unaccompanied by symptoms, except in the terminal phase of the disease. Intrathoracic manifestations are encountered in the form of pleural and pericardial effusions, fibrinous pericarditis, adhesions or fibrous thickening of the pleura, calcification over the surface of the pleura and epicardium with consequent restrictive lung disease, and constrictive pericarditis.
The pattern of involvement of mesothelial surfaces is similar in patients with Degos' disease and in some patients with lupus erythematosus.
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