In severe cases, the patient may need to be admitted for a diagnostic evaluation to determine the extent of disease and to exclude other vasculitides such as systemic lupus erythematosus (SLE) or polyarteritis nodosa (PAN). Malignant atrophic papulosis should be considered in patients diagnosed with a nonspecific vasculitis that does not respond to immunosuppressive therapy.
Admit the patient to the hospital immediately if a complication such as gastrointestinal bleeding, perforation, or stroke is suspected. Transfer may be required for surgical intervention in those patients who develop peritonitis or intracranial bleeding. (Surgical treatment usually is required for patients who develop complications such as gastrointestinal bleeding, intestinal perforation, bowel infarction, or intracranial bleeding.)
Broad-spectrum antibiotics may be used in patients with intestinal perforation in the perioperative period. Discontinuation of prothrombotic medications (eg, oral contraceptives) should be considered.
Monitor patients with isolated cutaneous disease for the development of systemic disease.
Because most patients initially present with skin manifestations, they typically are seen by a dermatologist, at which time a diagnosis usually is made. The skin lesions are not painful, usually do not itch, and generally do not require treatment. Patients who have gastrointestinal or neurologic symptoms should undergo an appropriate workup to detect systemic disease, which is an important determinant of prognosis.
Many medications have been tried for treatment of malignant atrophic papulosis, without consistent success. Degos suggested that anticoagulants might be effective, but others have shown them to be of no benefit. Isolated cases of benign cutaneous disease have responded to nicotine patches (5 mg/d transdermal patch) and pentoxifylline plus aspirin. Similarly, antiplatelet drugs, such as aspirin and dipyridamole, may reduce the number of new papules but have not shown any consistent benefit in systemic disease. Other drugs (eg, corticosteroids, immunosuppressants, sulfonamide, tetracycline, penicillin, interferon 2 alpha) have been shown to be ineffective in altering the course of the disease.
No special dietary or activity considerations are necessary.
Consider consultations with the following specialists: