[Intimal muscular fibrodysplasia responsible for an ischemic gastric ulcer in a patient with a von Recklinghausen's disease: a case report].
A 78 year-old woman, suffering from a von Recklinghausen's disease sought medical assistance for hematemesis with anemia. This patient had previously experienced an amputation of the right arm for gangrene. Gastric fibroscopy unveiled a deep chronic ulcus developed in the antrum, highly suspect of malignancy. Multiple biopsies of the ulcer showed mainly interstitial gastritis. The persistence of the hematemesis imposed a subtotal gastrectomy. Pathological examination of the operative specimen evidenced an ischemic ulcer caused by arterial intimal muscular fibrodysplasia with associated neurofibromatosis in the neighboring sub-mucosal layer. This case report highlights the frequent association of phacomatosis especially von Recklinghausen's disease, with vascular lesions whose clinical expression mainly depends on the involved vascular area.
Vuong
P N
PN
Unité d'Anatomie et de Cytologie Pathologiques, Hôpital Saint-Michel, 33 rue Olivier de Serres, Paris 75015 Paris, France.
Le Bourgeois
P
P
Houissa-Vuong
S
S
Martin
P
P
Berrod
J L
JL
D000368:Aged; D000740:Anemia / Q000209:etiology; D003937:Diagnosis, Differential; D005260:Female; D005352:Fibromuscular Dysplasia / Q000209:etiology* / Q000473:pathology; D006396:Hematemesis / Q000209:etiology; D006801:Humans; D007511:Ischemia / Q000209:etiology*; D009456:Neurofibromatosis 1 / Q000150:complications*; D011706:Pyloric Antrum / Q000098:blood supply*; D013274:Stomach Neoplasms / Q000175:diagnosis; D013276:Stomach Ulcer / Q000175:diagnosis / Q000209:etiology*; D017539:Tunica Intima / Q000473:pathology