Degos Disease Workup

Updated: Dec 10, 2019
  • Author: Meagan O Harris, MD; Chief Editor: Dirk M Elston, MD  more...
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Workup

Laboratory Studies

No specific laboratory test results are specific for a diagnosis of Degos disease; however, many patients present with abnormalities in clotting function. [17]

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Imaging Studies

No imaging studies are needed to evaluate the cutaneous lesions. If organ system involvement is suspected, appropriate imaging studies should be ordered.

Neurologic evaluation should include the following:

  • MRI of the brain to evaluate for cerebral infarctions or hemorrhage [43] and cord infarcts
  • MRI of the optic nerve [38]
  • Cerebral angiography, which can depict stenosis, ectasia, and aneurysms involving the peripheral branch of arteries [43]
  • Electroencephalogram testing - May show generalized nonspecific slowing
  • Electromyogram - May demonstrate axonal and demyelinating polyneuropathy

Cardiothoracic evaluation should include a chest radiograph.

Abdominal evaluation should include CT scanning of the abdomen/pelvis. [44]

 

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Other Tests

Endoscopy of the gastrointestinal tract (ie, stomach, esophagus, duodenum, colon, rectum) can show infarcted lesions or ulcers. Laparoscopy of the intestine can show a similar type lesions that manifest with white plaques with red borders on the serosal surface of the bowel and the peritoneum. Laparoscopy demonstrates superiority over endoscopy at detecting gastrointestinal involvement. [45]

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Histologic Findings

Early papules in Degos disease are skin colored and can demonstrate a superficial and deep perivascular, periadnexal, and perineural chronic inflammatory cell infiltrate associated with interstitial mucin deposition, as shown in the images below.

Superficial and mid-dermal perivascular lymphocyti Superficial and mid-dermal perivascular lymphocytic infiltrate with focal vacuolar change at the dermoepidermal junction (hematoxylin and eosin, X100). Courtesy of David F. Butler, MD.
Alcian blue stain for mucin (X100). Courtesy of Da Alcian blue stain for mucin (X100). Courtesy of David F. Butler, MD.

A mild vacuolar interface reaction has been described. The vacuolar interface dermatitis, dermal mucin, and perivascular lymphoid infiltrate mimic lupus erythematosus.

Fully developed papules can be raised, with umbilicated porcelain-white centers and a surrounding erythematous rim. Histologically, these papules demonstrate wedge-shaped degeneration of collagen, as shown in the image below.

Wedge-shaped area of dermal sclerosis (hematoxylin Wedge-shaped area of dermal sclerosis (hematoxylin and eosin, X100). Courtesy of David F. Butler, MD.

An interface dermatitis can be present but is often limited to the central portion of the tissue examined histologically. Additionally, squamatization of the dermoepidermal junction, melanin incontinence, and epidermal atrophy can manifest. [46]

In many cases, an area of papillary dermal sclerosis manifests that mirrors the early stages of lichen sclerosus et atrophicus.

Hyperkeratosis, epidermal atrophy, dermoepidermal separation, edema, and papillary dermal necrosis occur. Fibrinoid necrosis and thrombosis occur in the papillary dermis and in the capillary and venules. Others have noted that skin biopsy specimens show hyperkeratosis, atrophy of the epidermis, and necrobiosis of the collagen layer. Well-developed papules of Degos disease with epidermal atrophy and hyperkeratosis overlying a wedge-shaped area of cutaneous ischemia extending into the deep dermis have also been observed.

A superficial and deep perivascular lymphocytic infiltrate can gather at the fringe of ischemic areas. Marked endothelial swelling and occasional platelet-fibrin thrombi are often noted. Infarctive changes or scattered necrotic keratinocytes may be present in the epidermis. Abundant acid mucopolysaccharides often occur in the dermis, mimicking dermal mucinosis.

Direct immunofluorescence examination does not yield definitive results. Perivascular fibrin and complement can be present. In one reported case, autopsy results demonstrated the clinical diagnosis of Degos disease based on the finding of thromboangiitis of the Burger type, with bland infarcts of the small intestine and perforation of the jejunum.

In a case of Degos disease, Cavalié et al [35] demonstrated the use of reflectance confocal microscopy (RCM) to better characterize the pathognomonic telangiectatic ring viewed on dermoscopy. RCM revealed distinct features of the lesional and perilesional regions not previously reported. The telangiectatic ring corresponded to increased and dilated dermal capillaries.

Microaneurysms of the bulbar conjunctival vessels have been described.

Changes in the kidneys manifest with thickening of the afferent glomerular arterioles and the capillary basement membrane.

In 2008, Notash et al [36] described a fatal case of Degos disease in a 48-year-old man whose autopsy findings demonstrated diffuse fibrotic changes in the serosal membranes and the internal organs.

A case of the benign variant showing histopathologic similarities with lymphocytic vasculitis and lichen sclerosus has been described. Features mimicking lichen sclerosis included vacuolization of the dermoepidermal junction, keratinocyte necrosis, epidermal atrophy, and edema of the papillary and reticular dermis. [47]

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