Patients with malignant atrophic papulosis may have systemic involvement in multiple organ systems. Gastrointestinal (GI) involvement can cause bowel perforation. Central nervous system (CNS) involvement may put the patient at risk for stroke, intracranial bleeding, meningitis, and encephalitis.1,3 There can also be cardiopulmonary involvement that causes pleuritis and pericarditis.1 Ocular involvement can affect the eyelids, conjunctiva, retina, sclera, and choroid plexus.1 Renal involvement has been noted in a few cases.2
In a prospective, single-center cohort study of 39 patients with atrophic papulosis, systemic involvement (malignant atrophic papulosis) was reported in 29% (n = 11) of the patients.2 In these patients, involved organ systems included the GI tract (73%; n = 8), CNS (64%; n = 7), eye (18%; n = 2), heart (18%; n = 2), and lungs (9%; n = 1); 64% (n = 7) had multiorgan involvement. Mortality was reported in 73% of the patients with systemic disease.
Ongoing testing is required
For a patient presenting with atrophic papulosis, initial and follow-up visits should include evaluation for systemic manifestations through a full skin examination, fecal occult blood test, and ocular fundus examination.1,2 If the patient shows any symptoms that suggest systemic involvement, further testing is advised, including evaluation of renal function, colonoscopy, endoscopy, magnetic resonance imaging of the brain, an echocardiogram, and chest computed tomography.
Because internal organ involvement in malignant atrophic papulosis can develop within years of (benign) cutaneous manifestations, regular follow-up is recommended.1 Research suggests evaluation of patients with benign atrophic papulosis every 6 months for the first 7 years after disease onset and then yearly between 7 and 10 years after onset.2
Treatment options are limited
Antiplatelet agents (aspirin, pentoxifylline, dipyridamole, and ticlodipine) and anticoagulants (heparin) have led to partial regression of skin lesions in case reports.1 Some lesions seem to disappear after treatment, but due to limited evidence, it is difficult to determine whether treatment leads to a reduction of future lesions.
When it comes to malignant atrophic papulosis, there is no uniformly effective treatment. Antiplatelet agents and anticoagulants are often used as initial treatment, but efficacy has not been clearly demonstrated. In case reports, eculizumab and treprostinil have shown effectiveness in treating CNS involvement, but there are no uniform dosage recommendations.3,4
In this case, the patient had mild GI symptoms. A colonoscopy showed evidence of microscopic colitis, but there was no evidence of atrophic papulosis in the GI tract.
Additional laboratory work-up was ordered to evaluate for signs of organ involvement and to rule out any associated connective tissue disease or hypercoagulable state. Her results showed a mildly elevated erythrocyte sedimentation rate (29 mm/h) and a positive antinuclear antibodies assay (1:640, speckled pattern). She was referred to a rheumatologist, who found no evidence of a connective tissue disorder. A complete blood count, comprehensive metabolic panel, urinalysis, and hypercoagulability work-up were all within normal limits. A complete eye exam was also normal.
The patient was started on aspirin 81 mg/d. Because she continued to develop new lesions, her dermatologist added pentoxifylline extended release and gradually increased the dose to 400 mg in the morning and 800 mg in the evening. About 4 years after onset of the rash, the patient showed no signs of systemic involvement, but her skin lesions were still present.