[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.
Unité d'Anatomie et de Cytologie Pathologiques, Hôpital Saint-Michel, 33 rue Olivier de Serres, Paris 75015 Paris, France.
P N
PN
Vuong
P
P
Le Bourgeois
S
S
Houissa-Vuong
P
P
Martin
J L
JL
Berrod
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