Malignant atrophic papulosis

The lethal intestino-cutaneous syndrome which we described in 1942 as malignant atrophic papulosis (MAP) has gained various other visceral sites. However, the cutaneous eruption remains the constant and pathognomonic symptom, which, despite its benign appearance harbours a serious prognosis because of the frequently very severe lesions in the small intestine, and sometimes of the nervous system. The very special histological structure shows zones of necrosis (dermal in the skin) due to vasculitis with a tendency to thrombosis, affecting the small vessels below the lesion, and with little or no inflammatory reaction, which differentiates it from other angiitis. The aetiology remains uncertain (?viral) and the treatment is disappointing although heparin appears to have been helpful occasionally.