Malignant Atrophic Papulosis Workup

Updated: Apr 10, 2019
  • Author: L Campbell Levy, MD; Chief Editor: BS Anand, MD  more...
  • Print
Workup

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.

Next:

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 the 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.

Previous
Next:

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.

Previous
Next:

Procedures

Skin biopsy usually is required for histologic diagnosis.

Previous
Next:

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. [11]

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. [12]

Previous