An unusual presentation of giant cell arteritis.

A 77-year-old-man with giant cell arteritis who developed bitemporal scalp ulcerations is described. Since 1946 when Cooke et al. reported the first case of scalp necrosis there were approximately 55 cases published. Scalp ulceration is a rare complication of giant cell arteritis and occurs mainly in elderly persons, particularly women. About half of all patients were presented to dermatologists. Most of the patients (70%) had other serious complications of giant cell arteritis: blindness, gangrene of the tongue and nasal septum necrosis. Seventy percent of the cases were confirmed by a temporal artery biopsy. The necrosis were of varying extent and uni- or bilateral. Although, in most cases necrosis has been located bilaterally as in the presented case. Scalp healing was complete nearly in all patients by conservative treatment within a year. Scalp ulceration is a potentially reversible complication of giant cell arteritis which indicates extensive vessel involvement and adequate coricosteroid therapy is required and essential.

[Hypoplasia of the descending thoracic and abdominal aorta in Williams-Beuren syndrome]. / Hypoplasie der deszendierenden Aorta thoracalis und der Aorta abdominalis bei Williams-Beuren-Syndrom.

Hypoplasia of the descending thoracic and abdominal aorta is a very rare condition and its etiology is poorly understood. Associations with congenital and acquired disorders have been reported. In this article we present the case of a 24-year-old woman with hypoplasia of the thoracic and abdominal aorta and Williams-Beurensyndrome. This rare syndrome is attributed to deletions of genes on chromosome 7, among other the elastin-gene, and is characterized by cardiovascular anomalies, dysmorph facial features and mental retardation. The patient presented with a history of severe hypertension and recurrent abdominal pain since childhood. Diagnosis was established by duplex-sonography and magnetic resonance angiography. The patient was treated by an aortoaortic bypass from the ascending to the infrarenal aorta with reinsertion of the visceral and the right renal arteries. It is essential to recognize the condition early to withhold high morbidity and mortality resulting from long standing severe hypertension.

Acroparesthesia and acral arterial occlusions as first manifestations of essential thrombocythemia.

Thrombocytosis is either caused by a reactive process (secondary thrombocytosis) or by a clonal bone marrow disorder The latter category includes essential thrombocythemia with bleedings and thrombotic complications as major causes of illness and death in this patients. We describe a 43-year-old man with a 6 months history of acroparesthesia in his toes. Half a year after onset of these symptoms, he noticed a bluish discoloration of digit V of his left foot. On first presentation physical examination revealed a bluish discoloration of all toes and a cold and blue digit V of the left foot. Peripheral pulses were all palpable, normal ankle systolic pressure measurements and normal pulse volume recordings except for digit V of the left foot were found. Laboratory tests revealed thrombocytosis of 800000/microliter. On treatment with acetylsalicylacid, prostanoids intravenously and low molecular weight heparin, the patient became asymptomatic and pulse volume recording of digit V was normalized. After exclusion of cardial or vascular sources of embolism by utrasonography bone marrow aspirate and biopsy supported the diagnosis of essential thrombocythemia.