vascular complications in neurofibromatosis-1 ( nf-1 ) are not uncommon . however , many of these abnormalities may not be evident clinically . one of the causes for hypertension in these patients is renal artery stenosis , and the other is pheochromocytoma . a case of late onset nf-1 with bilateral partial radial artery stenosis resulting in digital gangrene is being reported . a 35-year - old male , product of a nonconsanguineous marriage , manual embroidery maker by profession , presented with painful ulcers involving thumb and index fingers of both hands for 3 months . he did not drink alcohol but was a smoker ( 3 - 4 cigarettes / day ) since 20 years . pulsations of the right radial artery could not be felt and left radial artery pulse was feebly felt . cutaneous examination revealed multiple neurofibromata and cafe au lait spots over back [ figure 1 ] . palmar freckling [ figure 2 ] and axillary freckling [ figure 3 ] were also seen . cafe au lait spots and cutaneous neurofibromata over patient 's back gangrene involving tip of thumbs and index fingers differential diagnoses included vascular nf-1 , thromboangitis obliterans ( tao ) , crest syndrome , and antiphospholipid syndrome ( aps ) . however , there was no history of pain at rest , intermittent claudication , and ischemic ulcerations . estimation of ana , anti phospholipid antibodies ( apla ) , cryoglobulin , and homocysteine levels could not be carried out as the patient was extremely poor . the patient was diagnosed to have vascular nf-1 with partial radial artery stenosis , after buerger 's disease , crest syndrome , and anti phospholipid syndrome were ruled out with available clinical findings and investigation results . he was treated with oral pentoxifylline ( 400 mg twice daily ) and aspirin ( 150 mg / day ) . he was advised to get regular ophthalmological examinations for early detection of a possible optic nerve pathway tumor . though not obvious clinically , vascular anomalies have been reported in nearly 50% of autopsies of patients suffering from nf-1 . apart from kidneys , vascular lesions are seen in intracerebral vessels , those of heart , endocrine glands , gastrointestinal tract , the pancreas , and spleen . these vascular changes are believed to be due to proliferation of schwann cells within arteries with secondary degenerative changes and fibrosis . the pathology of the lesions is divided into four types , namely ( 1 ) intimal , ( 2 ) advanced intimal proliferation occluding lumen , ( 3 ) aneurysmal form ( fibrohyaline thickening of intima with fragmentation of muscularis and elastica ) and ( 4 ) nodular form ( spindle and epithelioid cells disturbing integrity of vessel walls ) . two basic categories have also been described : one involving large vessels with surrounding neurofibromatous or ganglioneuromatous tissue , the other being dysplasia involving small vessels . in our patient , buerger 's disease ( tao ) was considered an important differential diagnosis . however , in color doppler , there was no thrombophlebitis as tao is a pan vasculitic condition . in tao , arteriography changes include thrombosis in the lumen , vessel being normal proximally , corkscrew collaterals and thrombophlebitis of adjacent veins . biopsy findings show luminal thrombosis with inflammatory infiltrate with intact internal elastic lamina . in nf-1 biopsy changes reported in nf-1 are as follows : adventitia surrounded by neurofibromatous or ganglioneuromatous tissue , thinning of media , fragmentation of elastic tissue , and intimal hyperplasia . severe systemic vasculopathy in a 4-year - old boy with nf-1 has also been reported . the aim of this report is to highlight various life - threatening vascular complications reported in nf-1 , to detect them early and treat them to prevent lethal complications .
not much attention is paid to vascular complications in neurofibromatosis-1 ( nf-1 ) though they are well known to occur . a case of vascular nf-1 with bilateral partial radial artery stenosis resulting in digital gangrene involving fingers of both hands is being reported here . 
 various vascular abnormalities in nf-1 as complications resulting from them and their management is briefly highlighted .