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Malignant Atrophic Papulosis Workup

  • Author: L Campbell Levy, MD; Chief Editor: BS Anand, MD  more...
 
Updated: Jun 17, 2016
 

Laboratory Studies

There are no laboratory results that are pathognomonic of malignant atrophic papulosis. Complete blood cell (CBC) count, serum chemistries, erythrocyte sedimentation rate (ESR), and C-reactive protein findings are usually within the reference ranges. Results of serum immunoglobulins, complement assays, antinuclear antibody (ANA), anti-double-stranded DNA (anti-dsDNA), and other serologies are usually unremarkable as well. Coagulation studies are generally normal. However, protein S deficiency, antiphospholipid antibodies, and altered platelet function have been identified in isolated cases of malignant atrophic papulosis, resulting in abnormalities of various coagulation parameters.

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Imaging Studies

In patients with neurologic involvement, computed tomography (CT) scanning or magnetic resonance imaging (MRI) of the brain may show ischemic infarcts, intracerebral bleeding, subdural hemorrhage, cord infarcts, and diffuse homogeneous dural enhancement. A cerebral angiogram may reveal narrowing and occlusion of small intracranial arteries. Generalized nonspecific slowing on EEG and axonal and demyelinating polyneuropathy on electromyogram (EMG) also have been found in selected patients.

In patients with abdominal discomfort and cutaneous malignant atrophic papulosis, plain radiographs, CT scan of the abdomen, or small bowel follow through may show intra-abdominal perforation, abscesses, or fistulae indicating systemic involvement.

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Other Tests

Gastrointestinal involvement may be observed on endoscopy, even in asymptomatic patients. Lesions similar to those on the skin are most often observed in the small bowel but can also be seen in the stomach, esophagus, duodenum, colon, and rectum.

Laparoscopy may show typical lesions consisting of white spots with hyperemic borders on the serosal surface of the bowel and the peritoneum.

Malignant atrophic papulosis infrequently causes symptomatic involvement of other organs (eg, lungs, heart), which may require appropriate tests such as chest x-ray, electrocardiography (ECG), and echocardiogram.

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Procedures

See the list below:

  • Skin biopsy usually is required for histologic diagnosis.
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Histologic Findings

Biopsy samples of early lesions have shown nonspecific findings, including some perivascular and perineural inflammatory infiltrates. However, a typical mature lesion of the skin usually shows an atrophic hyperkeratotic epidermis overlying an inverted, cone-shaped area of necrosis in the dermis. The small-caliber blood vessels in the dermis show narrowing of the lumen by endothelial proliferation and, sometimes, partial or complete occlusion of the lumen by a thrombus.

Although lesions may show lymphocytic perivascular infiltrates, it is the relative paucity or complete absence of inflammatory cells at the periphery of affected vessels that distinguishes malignant atrophic papulosis from other vasculitides. Similar changes are observed in the small arteries and arterioles on histologic examination of other affected organs. Although prominent IgA deposits have been reported in isolated cases, direct immunofluorescence has yielded variable results.[7]

A relatively recent in vivo skin imaging technique may provide more detailed histologic findings in malignant atrophic papulosis. Reflectance confocal microscopy (RCM) appears to have not only image resolutions similar to that of conventional microscopy (about 1 μm) but also a depth of up to 200 μm and a close correlation between RCM findings and underlying histologic changes.[8]

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Contributor Information and Disclosures
Author

L Campbell Levy, MD Fellow, Section of Gastroenterology and Hepatology, Department of Internal Medicine, Dartmouth Hitchcock Medical Center

L Campbell Levy, MD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

Coauthor(s)

Lawrence J Cheskin, MD Director, Johns Hopkins Weight Management Center; Associate Professor, Health, Behavior & Society, Johns Hopkins Bloomberg School of Public Health; Joint Appointment, Department of Medicine, Division of Gastroenterology, Johns Hopkins University School of Medicine; International Health/Human Nutrition, JH Bloomberg School of Public Health

Lawrence J Cheskin, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association

Disclosure: Received consulting fee from Medifast for board membership; Received none from Vivus for purchase of stock as an investment; Received none from Medifast for purchase of stock as an investment.

Brian E Lacy, MD, PhD Associate Professor of Medicine, Dartmouth Medical School; Director of GI Motility Laboratory, Department of Gastroenterology, Dartmouth Hitchcock Medical Center

Brian E Lacy, MD, PhD is a member of the following medical societies: Alpha Omega Alpha, American College of Gastroenterology, American Gastroenterological Association, American Neurogastroenterology and Motility Society, American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Douglas M Heuman, MD, FACP, FACG, AGAF Chief of Hepatology, Hunter Holmes McGuire Department of Veterans Affairs Medical Center; Professor, Department of Internal Medicine, Division of Gastroenterology, Virginia Commonwealth University School of Medicine

Douglas M Heuman, MD, FACP, FACG, AGAF is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Physicians, American Gastroenterological Association

Disclosure: Received grant/research funds from Novartis for other; Received grant/research funds from Bayer for other; Received grant/research funds from Otsuka for none; Received grant/research funds from Bristol Myers Squibb for other; Received none from Scynexis for none; Received grant/research funds from Salix for other; Received grant/research funds from MannKind for other.

Chief Editor

BS Anand, MD Professor, Department of Internal Medicine, Division of Gastroenterology, Baylor College of Medicine

BS Anand, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Additional Contributors

David Eric Bernstein, MD Director of Hepatology, North Shore University Hospital; Professor of Clinical Medicine, Albert Einstein College of Medicine

David Eric Bernstein, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

Julian Katz, MD Clinical Professor of Medicine, Drexel University College of Medicine

Julian Katz, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, American Gastroenterological Association, American Geriatrics Society, American Medical Association, American Society for Gastrointestinal Endoscopy, American Society of Law, Medicine & Ethics, American Trauma Society, Association of American Medical Colleges, Physicians for Social Responsibility

Disclosure: Nothing to disclose.

Acknowledgements

Robert J MacNeal, MD Staff Physician, Department of Dermatology, Critical Care Fellowship Reviewer, Dartmouth-Hitchcock Medical Center; Supervising Medical Officer, Veterans Administration Hospital, White River Junction, Vermont

Robert J MacNeal, MD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

Hemant Pande, MD Consulting Staff, Department of Gastroenterology, Leesville Surgical Clinic and Digestive Disease Center

Hemant Pande, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, and American Society for Gastrointestinal Endoscopy

Disclosure: Nothing to disclose.

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