AUTHOR AND EDITOR INFORMATION

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• Miscellaneous
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INTRODUCTION

Section 2 of 11  

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Background

In 1941, in an article entitled "Multiple Hautrekrosen bei Thromboangiitis obliterans," Kohlmeier described a case of a disease that has now been termed malignant atrophic papulosis (MAP) or Degos disease (DD). Degos recognized it as a distinct clinical entity in 1942.

For a gastroenterological perspective on MAP, see Malignant Atrophic Papulosis.

Broadly speaking, DD is a vasculopathy or an endovasculitis. It is an occlusive arteriopathy involving small-caliber vessels. Specifically, it is a progressive, small- and medium-size arterial occluding disease, leading to tissue infarction and initially involving the skin. DD occurs both in a limited benign, cutaneous form and in a lethal multiorgan, systemic variant.

In the skin, DD initially manifests with erythematous, pink or red papules. These papules heal to leave scars with pathognomonic, central, porcelain white atrophic centers. These papules usually have a peripheral telangiectatic rim.

In the systemic variant of DD, the gastrointestinal tract is affected in 50% of cases. Intestinal perforation is the most severe complication and the most common cause of death in systemic DD. Other systems can also be involved; approximately 20% of cases of systemic DD involve the CNS. Systemic manifestations usually develop from weeks to years after the onset of skin lesions, or, in rare instances, they may precede the skin lesions.

Because of the broad overlap in clinical and histological findings, High et al¹ contended in 2004 that DD may not be a specific entity but, rather, may represent a common end point to a variety of vascular insults, many of which have not been fully elucidated. In 2003, Ball et al² proposed that DD is just a variant of lupus.

In 2005, the author adduced strong evidence that DD is a distinct condition because, unlike lupus, (1) it does not involve the face, (2) it does not respond to therapies such as corticosteroids that at least abate lupus, (3) it does not manifest with photosensitivity, (4) viral inclusions are present in some cells in patients with DD, and (5) systemic DD is universally fatal, usually within 1-2 years, whereas lupus (even if severe) takes years to be fatal.³

Wilson et al⁴ reiterated that MAP/DD has both a (1) limited, cutaneous type and a (2) systemic, fatal variant.

Pathophysiology

The etiology and the pathophysiology of DD are unknown. Some have classified DD as a vasculitis, a mucinosis, or a thrombotic disorder. In most cases, no circulating immune complexes, antiendothelial cell antibodies, or anticardiolipin antibodies are isolated. Although, in some cases, antiphospholipid antibodies of uncertain significance are identified.

Some authorities suggest that DD involves a primary endothelial cell defect with secondary thrombosis, leading to infarctive changes. No evidence exists for antibodies to components of endothelial cells. Medications and toxic chemicals do not appear to induce DD.

The actual physical damage to blood vessels involves, at least in part, impaired fibrinolytic activity and alterations in platelet function. Classifying DD as a vasculitis may not be appropriate because inflammation of the vessel walls is minimal and because immune complexes have not been found in the vessel walls.

Three possible mechanisms for this pathology have been suggested: disturbance in immunity, viral infection, and abnormality in the clotting system of blood.

In familial cases, an autosomal dominant mode of inheritance has been suggested, but this is uncertain.

In molecular analysis of cases of DD with only cutaneous lesions, no paramyxovirus was identified by polymerase chain reaction.

Frequency

International

DD is rare. About 200 cases have been reported in the world literature.

Mortality/Morbidity

Systemic DD is frequently fatal within 2-3 years from the onset of systemic involvement. The cause of death is usually intestinal perforation. However, the range of survival time from time of diagnosis varies from less than 1 year to more than 12 years. Other causes of death include bowel infarction, pleuropericardial pathology, and neurologic infarction and hemorrhage.

• Wilson et al⁴ reviewed the 24 reported instances of MAP malignant/systemic type and benign/purely cutaneous type in pediatric patients. They found that 14 cases (58%) were fatal. Patients died an average of 3.6 years after the diagnosis of MAP.
• In 1 patient, in whom skin and abdominal symptoms occurred at the same time, death from bowel hemorrhage followed in 6 months.
• In 1996, Subbiah et al⁵ described the neurologic features of a series of 15 patients with DD at the Mayo Clinic. Each patient had the white papules that are the hallmark of DD (biopsy proven). Long-term follow-up revealed 6 patients were dead. Nine patients with skin lesions only were nearly asymptomatic. Immunosuppressive and antiplatelet agents did not halt disease progression. CNS infarcts and hemorrhages with intravascular thrombi and without evidence of vasculitis were notable findings at autopsy.
• Notash et al⁶ reported a 48-year-old Iranian man with lethal systemic DD.

Race

Some state that DD generally occurs in white young adults. However, DD is reported in blacks in Africa, Arabs in Jordan,⁷ Asians in Japan, and elsewhere. Any racial link is uncertain.

Sex

In 1997, Katz et al⁸ noted that the disorder usually occurs in young adults, and the male-to-female ratio is approximately 3:1.

Wilson et al⁴ reviewed benign cutaneous MAP in 34 men and women (30 adults and 4 kids) and noted that benign MAP is more commonly reported in women, at a female-to-male ratio of 3:1.

Age

All ages are affected. The fatal systemic variant of DD can occur in children. In 1999, Lankisch et al⁹ described a 16-year-old white adolescent girl with acute abdominal pain due to visceral involvement of DD that required extensive small-bowel resection. The skin manifestations had been present for 2 years before the correct diagnosis was made. She died as a result of CNS involvement from DD.

Jalil et al¹⁰ described a case in a 2-year-old child who presented with chronic abdominal pain of uncertain origin. 

The benign cutaneous variant of DD can occur in adults. Wilson et al⁴ looked at 34 patients with benign cutaneous MAP and found their average age was 37.6 years. In 1998, Farrell et al¹¹ described a case of a 44-year-old woman with DD and a lupus anticoagulant who, 4 years later, was alive and without systemic involvement. Electron microscopy of the white papules demonstrated interwoven tubular structures within the endothelial cells. This was consonant with reports in previous studies of DD. Farrell et al¹¹ thought that aspirin (300 mg/d) kept her cutaneous DD in check. In 1998, Requena et al¹² described a 58-year-old homosexual man with AIDS who developed typical cutaneous lesions of MAP, with no visceral involvement detected 2 years after the diagnosis of DD.

DD can occur in infants. DD has been reported in a 7-month-old girl who showed spontaneous aggregation of platelets. A good clinical response in this patient was obtained by treatment with aspirin and dipyridamole.¹³

CLINICAL

Section 3 of 11  

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History

In most cases, the first manifestation of DD is a rash. Some have stated that the disease involves the skin as the sole clinical manifestation in 37% of patients. The gastrointestinal tract is involved in about 50% of patients.

• Patients may have a family history of DD. In 1997, Katz et al⁸ described a familial variant of DD.
• DD associated with a spontaneous cure of diabetes has been reported.¹⁴
• Patients with DD may have a history of a variety of abdominal complaints, manifesting with abdominal pain, abdominal distention, cramps, nausea, vomiting, diarrhea, or constipation. Sometimes, patients may state that they experience weakness, fatigue, or weight loss, or symptoms of malabsorption.
• DD has been associated with systemic diseases. DD has occurred in patients with rheumatoid arthritis, HIV infection, and antiphospholipid antibodies and antiphospholipid syndrome.¹⁵
• Patients may have a history of a variety of neurologic complaints that include facial and acral paresthesia, headaches, dizziness, seizures, hemiplegia, aphasia, paraplegia, and gaze palsy.
• Patients may have a variety of eye problems, including diplopia, ptosis, and visual-field defects.
• Patients may experience weakness, shortness of breath, and chest pain.
• Inflammatory linear vasculopathy mimicking DD has been described. This is important to note because DD mimicking vasculitis has been described.
• Saglik et al¹⁶ noted a case of MAP with endocardial involvement and positive anticardiolipin antibodies.

Physical

The main physical finding of DD is a skin rash that manifests with porcelain white scars. DD also affects the eyes, the intestines, the brain, and other organs with a variety of physical findings.

• The most visible manifestation of DD is in the skin.
• DD manifests with (in most cases) multiple, small, red, raised papules that are 2-5 mm in diameter. After a few days, they enlarge and develop a central white spot that is depressed in comparison to the red skin around it. They heal, leaving depressed porcelain white scars with a rim of telangiectases. See Media Files 1-2.
• The papules predominantly occur on the trunk and the arms.
• Degos papules commonly manifest on the penis.⁶
• Degos more rarely occurs on the palms and soles.⁶
• One case has noted that Degos occurred on the scalp.
• No cases known to the author note DD-type papules on the face.
• In a 17-month-old child, progressive involvement of the fingers and the toes with torpid ulcers and apical necrotic amputations has been reported.
• Peristomal lesions have been reported.
• The rash of DD develops slowly and is usually mostly asymptomatic, but it may be accompanied by a slight burning sensation. The rash can arise anywhere except on the soles, the palms, and the face. The rash starts as pink or red papules that are 2-15 mm in diameter. The papules evolve into atrophic scars that are porcelain white. Sometimes, abdominal symptoms precede the rash, but this finding is uncommon.¹⁷
• A variety of ocular findings occur in DD.
• • Posterior subcapsular cataracts,¹⁸ visual field defects, ptosis, third cranial nerve palsies, blepharoptosis, and optic atrophy may be associated with DD. Optical neuritis, papilledema, and scleral plaques can be present.¹⁹
  • In 1986, Sibillat et al²⁰ reported that ophthalmologic symptoms were present in 35 of 105 extant reports of DD. DD manifested in the eye tissues, usually in the conjunctiva. The sclera, the episclera, the retina, the choroid, the optic nerve, and/or the neuro-ophthalmologic apparatus demonstrated damage consonant with DD.
• Constrictive pericarditis has been reported in DD. This condition might be induced by pericardial vasculitis, thereby causing the left ventricular wall motion abnormality.
• The lungs can be affected in DD. Pulmonary manifestations include pleuritis and bilateral pleural effusions.
• The liver and the kidneys may be involved and associated with a vasculitis.
• The brain and the nerves can be affected in DD.
• • In 1 patient with DD, the neurologic examination revealed a right-hemianopsia, paraparesis (with a sensory level at Th12), and a neurogenic bladder. In another patient, an ascending thoracic myelopathy was present.
  • In 1996, at the Mayo Clinic, Subbiah et al⁵ described a series of 15 patients. Ten patients developed neurologic manifestations. These findings included fatal hemorrhagic or ischemic strokes in 5, disabling polyradiculoneuropathy in 1, and nonspecific neurologic symptoms without objective findings in 4.
  • Other manifestations include strokes, headaches, epilepsy, or nonspecific neurologic symptoms (eg, memory loss, altered sensation).
• Patients experience abdominal pain. Gastrointestinal bleeding can result in vomiting blood or passing blood with bowel movements. Patients with DD may have enterocutaneous fistulae.

Causes

The cause of DD is unknown. Suggested causes include a virus, an immune defect, or a clotting defect.

DIFFERENTIALS

Section 4 of 11  

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Other Problems to be Considered

Primary ulceration of the small intestine
Crohn Disease
Atrophie blanche lesions in systemic lupus erythematosus
Dermal mucinosis

WORKUP

Section 5 of 11  

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Lab Studies

• No specific laboratory test can be used to aid in diagnosing DD. In fact, most laboratory test results are normal, with the exception of the manifestation of anemia secondary to intestinal bleeding.
• In 1 patient, laboratory examinations disclosed persistent elevations of the thrombin-antithrombin III complex, plasmin-alpha 2 plasmin inhibitor complex, and cytotoxic T-cell subset (CD8⁺CD11^-), illustrating the coagulative, fibrinolytic, and immunologic implications of DD.
• In a series of 3 patients with DD, prolonged euglobulin lysis time, increased plasminogen activator levels, and increased plasminogen activator inhibitor activities were detected before and after a venous occlusion test, indicating an inhibition of fibrinolysis.
• In a series of 3 patients with DD, electron microscopy demonstrated an increased number of Weibel-Palade bodies and a raised staining of von Willebrand factor in endothelial cells in 1 patient.
• In a series of 3 patients, test results for coagulation and circulating anticoagulant were in the reference range.
• In a series of 3 patients, the results of platelet adhesion showed decreased adhesion in 1 patient and increased adhesion in another patient. Platelet aggregation study results decreased to the reference range in 2 patients. In one patient, tests showed hyperactive spontaneous and induced platelet aggregation.
• Amounts of protein in the cerebrospinal fluid and amounts of platelet aggregation have been reported as sharply increased in progressive stages of the disease. For example, laboratory results showed a gradual increase of cerebrospinal fluid proteins (from 156 mg/dL to 602 mg/dL) and an extremely increased amount of platelet aggregation.
• In DD, viruslike inclusions are frequently present in the endothelial cells and fibroblasts. C3 deposits and the presence of intracytoplasmic cylinders are also frequently present in the histiocytes.
• Hohwy et al²¹ reported a fatal case of systemic MAP in a man with factor V Leiden mutation and lupus anticoagulant.

Imaging Studies

• MRI of the brain can show multiple cerebral infarctions accompanied by small hemorrhagic areas and gadolinium-diethylenetriamine pentaacetic acid enhancement of the dura.²² They can also reveal intracerebral bleeding, subdural hemorrhage, and cord infarcts.
• A cerebral angiogram may reveal narrowing and occlusion of small intracranial arteries. Specifically, a cerebral angiogram can depict stenosis, ectasia, and aneurysms involving the peripheral branch of arteries.²²
• EEG tests have shown generalized nonspecific slowing.
• In some patients, electromyograms demonstrate axonal and demyelinating polyneuropathy.
• With angiograms, stenoses can be observed in the celiac artery and the small arteries in the kidney.
• In some patients, chest radiographs have depicted extensive calcification of the pericardium. Although pleural involvement and pericardial involvement have been reported in DD, constrictive pericarditis is most unusual, and radiographically demonstrable calcification of the pericardium has not been previously reported.
• Matsuura et al¹⁹ noted a fatal case of MAP involving the optic nerve and spinal cord. MRI of the optic nerve demonstrated (1) pathologic signal enhancement on fat-suppressed, T1-weighted images after intravenous meglumine gadopentetate infusion and (2) a sawtooth pattern over 7 vertebral segments of the spinal cord on T2-weighted sagittal images. These findings were confirmed at autopsy, during which pathologists grossly noted (1) a pronounced decrease of myelinated nerve fibers in the left optic nerve with thrombotic obstruction of the central retinal artery, (2) spongy degeneration in all levels of the spinal cord, and (3) patchy and moth-eaten patterns due to thromboses and endothelial proliferation in subarachnoid vessels.
• Amaravadi et al²³ described CT scan findings a 40-year-old woman. CT scanning of the abdomen and pelvis with oral and intravenous contrast demonstrated extensive ascites and nodular thickening of the omentum. A subsequent CT scan of the abdomen and pelvis with intravenous contrast showed a large amount of ascites with small bowel wall thickening and patchy mucosal perfusion and intraluminal hemorrhage into a loop of infarcted jejunum, which was later confirmed at autopsy to be an intraluminal clot. A final CT scan of the abdomen and pelvis with only intravenous contrast, which was performed to investigate worsening peritonitis, showed extensive pathology. An axial image demonstrated pneumoperitoneum with edematous and hyperemic small bowel loops consistent with small bowel ischemia. Discontinuity in the wall of a loop of jejunum was consistent with perforation. Multiple jejunal perforations were later demonstrated at autopsy.

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 similar type lesions that manifest with white plaques with red borders on the serosal surface of the bowel and the peritoneum.
• In 2007, Amaravadi et al²³ reported autopsy findings from a 40-year-old female DD patient. They found omental infarction and multiple yellow plaques on the serosal surface of the small bowel during an exploratory laparotomy.

Histologic Findings

Early papules in DD are skin colored and can demonstrate a superficial and deep perivascular, periadnexal, and perineural chronic inflammatory cell infiltrate associated with interstitial mucin deposition. See Media Files 3-4.

Biopsy results from the epidermis of the papules of DD can show a mild vacuolar interface reaction, and, at this early phase, the histologic appearances of DD can mimic tumid 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. See Media File 5. 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.²⁴

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 DD 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 DD based on the finding of thromboangiitis of the Burger type, with bland infarcts of the small intestine and perforation of the jejunum.

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 al⁶ described a fatal case of DD in a 48-year-old man whose autopsy findings demonstrated diffuse fibrotic changes in the serosal membranes and the internal organs.

TREATMENT

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Medical Care

No successful medical therapy for DD is known. Antiplatelet drugs (eg, aspirin, dipyridamole) may reduce the number of new lesions in some patients with only skin involvement. Some believe intravenous immunoglobulin may have a role in treatment.²⁵ Other treatments that have been tried without real effect include the following: topical corticosteroids, phenformin and ethylestrenol, iodohydroxyquinoline, aspirin and dipyridamole, phenylbutazone, arsenic, sulphonamides, dextran, corticosteroids, heparin, warfarin, niacin, streptomycin, corticotropin, azathioprine, methotrexate, cyclosporine, tacrolimus, mycophenolate mofetil, pentoxifylline, and clopidogrel.

Hohwy et al²¹ treated a patient with fatal systemic MAP with narrow-band ultraviolet (UVB) light and prednisolone and, later, with aspirin, pentoxifylline, and warfarin. Treatment was not effective. Interestingly, the patient has a mutation of factor V Leiden and lupus anticoagulant.

Surgical Care

When gastrointestinal bleeding, intestinal perforation, bowel infarction, or intracranial bleeding occurs, proper surgical intervention is necessary.

Consultations

Consult the following physicians:

• Dermatologists
• Gastroenterologists
• Neurologists
• General surgeons
• Neurosurgeons
• Ophthalmologists

Activity

Patients are often weak and this limits their activity, but no limitation to DD itself exists.

MEDICATION

Section 7 of 11  

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Medical therapy has no clear role in the treatment of DD. Some authorities have used antiplatelet medications, but their ultimate effect is unclear.

FOLLOW-UP

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Further Inpatient Care

• Care is primarily supportive. Admission to the hospital can be necessary in cases of gastrointestinal bleeding or other systemic complications.

Further Outpatient Care

• Patients should be monitored for systemic manifestations of DD and treated accordingly.

In/Out Patient Meds

• The role of medications is unclear. Patients might need transfusions if intestinal bleeding is present and, antibiotics, if an abdominal perforation is present.

Transfer

• Patients who manifest with systemic symptoms must be transferred to a center that can provide appropriate care.

Complications

• Complications of DD include the following:
• • Gastrointestinal bleeding and perforation
  • Neurologic bleeding and collapse
  • Cardiac pathologic conditions
  • Pulmonary pathologic conditions
  • Ocular complications

Prognosis

• Patients with only skin lesions may have a good prognosis.
• Gastrointestinal involvement may occur in as many as 60% of patients, and death in such cases is likely.
• About 15% of patients with DD have long-term survival, with disease often limited to the skin and with no manifestations of fatal bowel or CNS involvement.

Patient Education

• Because systemic DD is fatal, patients and their families should be educated in this regard.

MISCELLANEOUS

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Medical/Legal Pitfalls

• Failure to diagnosis the condition is a pitfall. Although the treatment of systemic DD does not change its disease course or cure DD, treatment of gastrointestinal and neurologic complications prolongs the patient's life.

Special Concerns

• DD can occur in infants. DD has been reported in a 7-month-old girl who showed spontaneous aggregation of platelets. A good clinical response in this patient was obtained by treatment with aspirin and dipyridamole.
• In patients with benign cutaneous DD, immunosuppression (in 1 patient with cyclosporine) can cause DD to evolve into systemic fatal disease.

MULTIMEDIA

Section 10 of 11  

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Media file 1:  Four papules of Degos disease located on upper-inner arm in different stages of evolution. Courtesy of David F. Butler, MD.
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Media file 2:  Papule of Degos disease with central sclerosis and telangiectasia. Courtesy of David F. Butler, MD.
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Media file 3:  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.
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Media type:  Histology

Media file 4:  Alcian blue stain for mucin (X100). Courtesy of David F. Butler, MD.
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Media type:  Histology

Media file 5:  Wedge-shaped area of dermal sclerosis (hematoxylin and eosin, X100). Courtesy of David F. Butler, MD.
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Media type:  Histology

REFERENCES

Section 11 of 11

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2. Ball E, Newburger A, Ackerman AB. Degos' disease: a distinctive pattern of disease, chiefly of lupus erythematosus, and not a specific disease per se. Am J Dermatopathol. Aug 2003;25(4):308-20. [Medline].
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21. Hohwy T, Jensen MG, Tøttrup A, Steiniche T, Fogh K. A fatal case of malignant atrophic papulosis (Degos' disease) in a man with factor V Leinden mutation and lupus anticoagulant. Acta Derm Venereol. 2006;86(3):245-7. [Medline].
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23. Amaravadi RR, Tran TM, Altman R, Scheirey CD. Small bowel infarcts in Degos disease. Abdom Imaging. Apr 10 2007;[Medline].
24. Scheinfeld N. Malignant atrophic papulosis. Clin Exp Dermatol. Sep 2007;32(5):483-7. [Medline].
25. Zhu KJ, Zhou Q, Lin AH, Lu ZM, Cheng H. The use of intravenous immunoglobulin in cutaneous and recurrent perforating intestinal Degos disease (malignant atrophic papulosis). Br J Dermatol. Jul 2007;157(1):206-7. [Medline].
26. Bogenrieder T, Kuske M, Landthaler M, Stolz W. Benign Degos' disease developing during pregnancy and followed for 10 years. Acta Derm Venereol. 2002;82(4):284-7. [Medline].
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Article Last Updated: Feb 14, 2008