No abstract

This 44-year-old man with Ehlers-Danlos syndrome (EDS) Type IV presented with hemiparesis and the Gerstmann syndrome. Left carotid artery (CA) angiography revealed a dissecting aneurysm with severe stenosis located in the common CA; the lesion was successfully treated with a stent graft. The patient's clinical course after endovascular surgery was uneventful, without occurrence of megacolon. The literature for spontaneous CA dissection in EDS Type IV cases is reviewed and points for investigation and treatment are discussed.

We investigated the differences between elderly and under-65-year-old patients using the psychiatric emergency system. The following characteristics were more common in elderly patients than in younger patients: organic mental disorder, mood disorder, dementia, disturbed consciousness, no excitation, physical complications, no history of visiting a psychiatrist and no history of hospitalization. In addition, significantly more elderly patients with mood disorder attempt suicide.

We report 45- and 61-year-old women with generalized prurigo nodularis-like eruption whose clinical, histologic and immunopathologic features were consistent with the diagnosis of pemphigoid nodularis. In one case, nodular lesions preceded the onset of generalized blistering by two years and in the other, no definite blister nor erosion was seen except for some appearing on the soles during the course of the disease. Western immunoblotting of EDTA-separated epidermal extracts revealed that the 230-kD bullous pemphigoid (BP) antigen was recognized by circulating autoantibodies in the patient sera, but the 180-kD BP antigen was not. The 180-kD BP antigen was recognized weakly by immunoblotting of the 180-kD BP antigen NC16a domain fusion protein, which shows high detection sensitivity. These findings suggested that weak reactivity of autoantibodies with either whole or a part of the 180-kD BP antigen molecule in some way accounts for negligible or localized blister-formation in this disorder. However, no particular change was noted in the reactivity with 180-kD BP antigen between the patient serum obtained before and after the development of generalized blistering. It is possible that different factors from the changes in serum reactivity with BP antigens may be involved in initiating generalized blistering.

Annular erythema developed in 22 patients with Sjögren syndrome. Clinically, the annular erythema was subdivided into three forms: Sweet disease-like annular erythema with an elevated border (14 cases); subacute cutaneous lupus erythematosus (SCLE)-like marginally scaled erythema (5 cases); and papular erythema (3 cases). Histopathologically, features commonly seen in annular erythema are deep perivascular and/or periappendageal infiltration of the lymphocytes with an admixture of neutrophils or plasma cells and less frequent epidermal changes suggestive of cutaneous lupus erythematosus. Immunoglobulin or complement deposition along the dermoepidermal junction of lesional skin was observed in 8 of 18 cases, and most of the dermal infiltrates consisted of CD4(+), 4B4(+) cells. The appearance of anti-SS-A(Ro) (100%) and anti-SS-B(La) (77%) was significantly higher in patients with annular erythema. These results suggest that patients with Sjögren syndrome might have a distinct annular erythematous lesion that is both clinically and histopathologically different from SCLE, although close immunologic abnormalities exist in these two diseases.