ABSTRACT
In generalized amyloidosis, involvement of heart is not rare, but development fo severe conduction distubance in cardiac amyloidosis is a rare presentation in a clinical situation. We report the first case in Korea of cardiac amyloidosis with complete atrioventricular block, which was confirmed by endomyocardial biopsy. A 59-year-old woman was admitted due to drowsy mentality. We observed a severe atrioventricular conduction disturbance on the electrocardiogram and a marked hypertrophied ventricular wall and a granular sparkling appearance within the wall, a characteristic feature in cardiac infiltration of amyloidosis, on the echocardiography. Endomyocardial biopsy was performed and revealed extensive deposition of amorphous eosinophilic materials within the cardiac muscle cells. Polarizing illumination after Congo-red staining showed typical green birefrigence. We performed a insertion of permanent pacemaker(VVI type) for the treatment of complete atrioventricular block.
Subject(s)
Female , Humans , Middle Aged , Amyloidosis , Atrioventricular Block , Biopsy , Echocardiography , Electrocardiography , Eosinophils , Heart , Korea , Lighting , Myocytes, CardiacABSTRACT
We report a case of epidermolysis bullosa acquisita in a 53 year-old woman who had. extreme skin fragility, trauma induced blisters and erosions usually localized to extensor site of the skin surface, and healing with scars and milia for several years. Histologic findings of perilesional skin showed normal epidermis, subepid malblister and spirsely infiltration of inflammatory cells. Direct immuncofluorescence showed depositiori of IgCi and C3 in a linear pattern along the epidermal basement, membrane in the per ilesional skin. In indirect immuriofluorescence, using 1.0M sodium chloride separated nor mal human skin as the sntbstrate, antitodies(IgG at a titer of 1:40) were bound to dermal site of the separation, indicating that the patient serum yields the reaction in or below the lamiria densa. Electronmicroscopic findings showed the roof of the blister was located. below the basal lamiria.