eMedicine Specialties > Gastroenterology > Systemic Disease

Malignant Atrophic Papulosis: Differential Diagnoses & Workup

Author: L Campbell Levy, MD, Fellow, Section of Gastroenterology and Hepatology, Department of Internal Medicine, Dartmouth Hitchcock Medical Center
Coauthor(s): 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; Brian E Lacy, MD, PhD, Associate Professor of Medicine, Dartmouth Medical School; Director of GI Motility Laboratory, Department of Gastroenterology, Dartmouth Hitchcock Medical Center; Hemant Pande, MD, Consulting Staff, Department of Gastroenterology, Leesville Surgical Clinic and Digestive Disease Center; Lawrence Cheskin, MD, Chief, Associate Professor, Department of Medicine, Division of Gastroenterology, Bayview Medical Center, Johns Hopkins University School of Medicine
Contributor Information and Disclosures

Updated: Aug 29, 2006

Differential Diagnoses

Crohn Disease
Polyarteritis Nodosa
Systemic Lupus Erythematosus
Tuberculosis

Other Problems to Be Considered

Thromboangiitis obliterans
Lichen planus
Antiphospholipid antibody syndrome
Lymphomatoid papulosis
Arthropod bite
Atrophie blanche
Tumid lupus

Workup

Laboratory Studies

  • There are no lab results pathognomonic of MAP. CBC, serum chemistries, erythrocyte sedimentation rate (ESR), and C-reactive protein findings are usually within 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 MAP, resulting in abnormalities of various coagulation parameters.

Imaging Studies

  • In patients with neurological involvement, CT scan or 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 MAP, plain radiographs, CT scan of the abdomen, or small bowel follow through may show intra-abdominal perforation, abscesses, or fistulae indicating systemic involvement.

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

Procedures

  • Skin biopsy usually is required for histologic diagnosis.

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. While 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 MAP 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.

More on Malignant Atrophic Papulosis

Overview: Malignant Atrophic Papulosis
Differential Diagnoses & Workup: Malignant Atrophic Papulosis
Treatment & Medication: Malignant Atrophic Papulosis
Follow-up: Malignant Atrophic Papulosis
References
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Further Reading

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Keywords

MAP, Kohlmeier-Degos syndrome, Degos disease, vascular occlusive disorders, thromboangiitis obliterans

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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)

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.

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 Motility Society, and American Society for Gastrointestinal Endoscopy
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.

Lawrence Cheskin, MD, Chief, Associate Professor, Department of Medicine, Division of Gastroenterology, Bayview Medical Center, Johns Hopkins University School of Medicine
Lawrence Cheskin, MD is a member of the following medical societies: American College of Gastroenterology, American College of Physicians, and American Gastroenterological Association
Disclosure: Nothing to disclose.

Medical Editor

David Eric Bernstein, MD, Chief, Section of Hepatology, North Shore University Hospital, Director, Associate Professor, Department of Internal Medicine, Division of Hepatology, New York University School 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, and American Society for Gastrointestinal Endoscopy
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Douglas M Heuman, MD, FACP, Director of Hepatology, McGuire Veterans Affairs Medical Center, Professor, Department of Internal Medicine, Division of Gastroenterology, Virginia Commonwealth University School of Medicine
Douglas M Heuman, MD, FACP is a member of the following medical societies: American Association for the Study of Liver Diseases, American College of Physicians, and American Gastroenterological Association
Disclosure: Nothing to disclose.

CME Editor

Alex J Mechaber, MD, FACP, Assistant Dean for Medical Curriculum, Associate Professor of Medicine, Division of General Internal Medicine, University of Miami Miller School of Medicine
Alex J Mechaber, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, and Society of General Internal Medicine
Disclosure: Nothing to disclose.

Chief Editor

Julian Katz, MD, Clinical Professor of Medicine, Drexel University College of Medicine; Consulting Staff, Department of Medicine, Section of Gastroenterology and Hepatology, Hospital of the Medical College of Pennsylvania
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 and Ethics, American Trauma Society, Association of American Medical Colleges, and Physicians for Social Responsibility
Disclosure: Nothing to disclose.

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