Sujet(s)
Acitrétine/administration et posologie , Femelle , Humains , Adulte d'âge moyen , Pemphigus chronique bénin familial/traitement médicamenteux , Pemphigus chronique bénin familial/épidémiologie , Pemphigus chronique bénin familial/anatomopathologie , Dermatoses vésiculobulleuses/épidémiologieRÉSUMÉ
Scleroderma renal crises (SRC) is a serious complication of systemic sclerosis whose prognosis remains serious despite management with angiotensin-converting enzyme inhibitors, antihypertensives and dialysis. Pulmonary renal syndrome (PRS), characterised by diffuse alveolar hemorrhage (DAH) and SRC, is rare and carries a grave prognosis. This case report discusses the clinicopathological features of a 43-year-old male presenting with severe hypertension and rapidly progressive renal failure who subsequently developed DAH and died. The clinical course, exhaustive investigative work-up and autopsy findings led to a diagnosis of diffuse systemic sclerosis with PRS subcategorized into PRS with thrombotic microangiopathy. The index case came without a prior diagnosis of systemic sclerosis, thereby posing a serious diagnostic challenge and management issues.
RÉSUMÉ
Dieulafoy disease is a rare cause of severe gastrointestinal haemorrhage. The lesion is usually located in the stomach, although it may occur anywhere in the gastrointestinal tract. It is characterized by severe bleeding from a minute submucosal arteriole that bleeds through a punctate erosion in an otherwise normal mucosa. We describe a 55 year old patient of severe upper gastrointestinal bleeding caused by a gastric Dieulafoy disease, presenting as a mass lesion in the stomach, diagnosed clinically and radiologically as gastrointestinal stromal tumour. Increased awareness and early endoscopy are important for accurate diagnosis.