Late manifestation of a fatal Behçet's disease with cardiac involvement and lethal outcome

Deutsche medizinische Wochenschrift
M KaatzU Wollina

Abstract

One year before admission a 67-year-old man of German origin developed extensive ulcerations of the limbs, later also of the trunk and neck. In addition to recurrent oral aphthous ulcers it was associated with erythema nodosum and intermittent arthritis of the elbow knee and ankle joints. During the last month he also had dyspnoea. Echocardiography revealed a dilated cardiomyopathy (DCM). He was admitted because of the progressive skin disorder. Physical examination showed a disoriented man with dyspnoea, cyanosis of the lips and pretibial oedema. He had aphthous ulcers in the mouth and on the genitals. Adamant Behçet disease was suspected. Erythrocyte sedimentation rate was raised to 21/48 mm/h, white cell count to 15,800/microliter. Tests for HLA B5 and B27 were negative, skin biopsy revealed superficial necrotizing vasculitis of postcapillary venules. Initially high doses of prednisolone, 100 mg/d intravenously) and azathioprine (100 mg/d orally) were administered, and within a few days both the skin and cardiac changes had regressed. Prednisolone dosage was reduced and cyclosporin (350 mg/d) substituted for azathioprine. He was discharged in a markedly improved general condition and with only a few tibial ulcerations. 4 mon...Continue Reading

Citations

May 30, 2002·AJR. American Journal of Roentgenology·Josée DuboisJulie Powell
Oct 16, 2009·Clinical Rheumatology·Yusuf SezenRamazan Geyik
Apr 9, 2010·International Journal of Rheumatic Diseases·Mustafa Al IzziMohammed Arif
May 20, 2015·Intractable & Rare Diseases Research·Selami DemirelliArif Arisoy
Feb 20, 2021·Journal of Experimental Pharmacology·Burçin Cansu Bozca, Erkan Alpsoy

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