**4.11 Malignant atrophic papulosis (Köhlmeier-Degos disease)**

Malignant atrophic papulosis (MAP) is an obliterating endovasculitis of smalland medium-sized arteries that produces tissue infarction as its main feature. It is considered an uncommon disease of unknown cause and can affect the skin, gastrointestinal tract and central nervous system, and the involvement of these last two systems can be fatal [40].

There is no fully effective treatment for the disease. Some authors use acetylsalicylic acid (300 mg daily) and/or dipyridamole (75 mg twice daily) as the first therapeutic modality, which facilitates blood perfusion. Other therapeutic options such as aspirin, heparin and warfarin can be used, however, aspirin is more associated with resurgence of lesions when discontinuing the drug [41].

More recently, studies have been conducted using eculizumab that have shown initial efficacy in skin and intestinal lesions, but the drug has not been able to prevent the development or progression of systemic manifestations. Subcutaneous treprostinil has been successfully tested in some cases with dramatic and sustained improvement in clinical status, although the response was not immediate. The mechanism of action of treprostinil in this scenario is not yet well understood [42, 43].

The use of corticosteroids, chloroquine or other immunosuppressants has proved unsatisfactory and has great potential to worsen the disease by unknown mechanism; therefore, they are not indicated [43].

### **4.12 Superficial thrombophlebitis**

These are vascular inflammations with thrombus formation and consequent occlusion or may occur due to slow flow within a varicose vein. If thrombophlebitis is found in apparently normal superficial veins, attention should be paid to the possibility of underlying malignancy, thrombosing coagulopathy and silent deep vein thrombosis [44].

For therapeutic management, in cases of limited superficial thrombophlebitis below the knee, without evidence of deep vein thrombosis, compression by specific stockings and the use of nonsteroidal anti-inflammatory drugs are enough, providing symptomatic relief. However, if there is deep venous thrombosis or extension to the saphenofemoral or saphenopopliteal junctions, prophylactic use of low molecular weight heparin may be necessary [44].
