[Scleroderma and Rendu-Osler disease. A casual association? (author's transl)].
An uncommon association of scleroderma and Rendu-Osler disease in the same patient is reported. The authors have not found similar descriptions after an extense bibliographic review. A possible relationship is suggested on the basis of the etiopathogenesis of both disorders; Rendu-Osler syndrome is an hereditary disease of unknown etiology with mesoblastic involvement (embryonic tissue which gives origin to the connective tissue), and scleroderma is an immune disease characterized by diffuse sclerosis of the connective tissue. Rendu-Osler disease was diagnosed because of the presence of angiomatous telangiectasias since childhood, recurrent mucosal hemorrhages, and the possible existence of the syndrome in a familial member. The diagnosis of scleroderma was supported by the demonstration of: 1) cutaneous infiltration, 2) Raynaud's phenomenon, 3) functional esophageal alterations with clinical dysphagia, 4) resorption of the distal phalanges, and 5) conclusive skin biopsy.
D000368:Aged; D005260:Female; D006225:Hand / Q000000981:diagnostic imaging / Q000473:pathology; D006801:Humans; D008046:Lip / Q000000981:diagnostic imaging / Q000473:pathology; D011859:Radiography; D012595:Scleroderma, Systemic / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology
Esclerodermia y enfermedad de Rendu-Osler: una asociación casual?