Metastatic Crohn disease: a review of dermatologic manifestations and treatment.

It is estimated that almost half of patients affected with Crohn disease (CD) experience a dermatologic manifestation of the condition. Metastatic CD (MCD) is a rare dermatologic entity, with as few as 100 cases reported in the literature. As such, MCD presents a clinical dilemma in diagnosis and management. The etiology of MCD is not well defined; however, prevailing theories agree that the underlying mechanism is an immunologic response to gut antigens. Diagnosis requires a high index of suspicion and usually is made by exclusion of other processes. Treatment success has been reported with the use of antibiotics, immunosuppressants, and sometimes surgical treatment. We review the etiology/epidemiology, diagnostic criteria, and treatment of this uncommon condition.

Accumulation of Ubiquitin and Sequestosome-1 Implicate Protein Damage in Diacetyl-Induced Cytotoxicity.

Inhaled diacetyl vapors are associated with flavorings-related lung disease, a potentially fatal airway disease. The reactive α-dicarbonyl group in diacetyl causes protein damage in vitro. Dicarbonyl/l-xylulose reductase (DCXR) metabolizes diacetyl into acetoin, which lacks this α-dicarbonyl group. To investigate the hypothesis that flavorings-related lung disease is caused by in vivo protein damage, we correlated diacetyl-induced airway damage in mice with immunofluorescence for markers of protein turnover and autophagy. Western immunoblots identified shifts in ubiquitin pools. Diacetyl inhalation caused dose-dependent increases in bronchial epithelial cells with puncta of both total ubiquitin and K63-ubiquitin, central mediators of protein turnover. This response was greater in Dcxr-knockout mice than in wild-type controls inhaling 200 ppm diacetyl, further implicating the α-dicarbonyl group in protein damage. Western immunoblots demonstrated decreased free ubiquitin in airway-enriched fractions. Transmission electron microscopy and colocalization of ubiquitin-positive puncta with lysosomal-associated membrane proteins 1 and 2 and with the multifunctional scaffolding protein sequestosome-1 (SQSTM1/p62) confirmed autophagy. Surprisingly, immunoreactive SQSTM1 also accumulated in the olfactory bulb of the brain. Olfactory bulb SQSTM1 often congregated in activated microglial cells that also contained olfactory marker protein, indicating neuronophagia within the olfactory bulb. This suggests the possibility that SQSTM1 or damaged proteins may be transported from the nose to the brain. Together, these findings strongly implicate widespread protein damage in the etiology of flavorings-related lung disease.

Vascular geometry of the extracranial carotid arteries: an analysis of length, diameter, and tortuosity.

BACKGROUND: Stable access is essential for successful intracranial interventions. Quantifying variations in extracranial carotid arteries may help in the selection and development of access catheters. This study describes the vascular dimensions from the aortic arch to the skull base. METHODS: CT angiography analysis was performed on 100 patients. The lengths, diameters, and tortuosity of the common carotid artery (CCA) and internal carotid artery (ICA) were measured from the aortic arch to the skull base. RESULTS: The mean±SD length of the carotid artery from the aortic arch to the skull base was 22.2±2.2 cm for the right side and 20.8±1.9 cm for the left side (p<0.0001). The length of the right CCA was 13.6±1.2 cm and the length of the left CCA was 12.4±1.4 cm (p<0.0001). The length of the right ICA was 8.6±1.4 cm compared with 8.4±1.4 cm for the left ICA (p=0.3). The ICA length in men and women was 8.9±1.3 cm and 8.2±1.3 cm, respectively (p=0.0001), and the CCA length in men and women was 13.6±1.5 cm and 12.3±1.6 cm, respectively (p<0.0001). The lengths of the CCA and ICA in patients aged ≥60 years were 13.3±1.7 cm and 8.9±1.5 cm, respectively compared with 12.8±1.7 cm and 8.2±1.1 cm, respectively, for patients aged <60 years (p=0.04 for CCA, p=0.0002 for ICA). Tortuosity of the CCA and ICA was 1.2±0.2 and 1.3±0.1, respectively, in patients aged ≥60 years compared with 1.1±0.1 for both the ICA and CCA in patients aged <60 years (p<0.0001 for both). There was a consistent ratio of CCA/ICA length of 1.6±0.3 on the right and 1.5±0.3 on the left (p<0.0001). The arterial diameters did not show any significant difference. CONCLUSIONS: The distance from the aortic arch to the skull base is longer on the right than on the left side. Both the CCA and ICA are longer in men and in patients aged ≥60 years. The tortuosity of both segments significantly increases with age.

Myeloid Sarcoma of the Bladder in the Setting of Refractory Anemia with Excess Blasts-2 (RAEB-2).

Myeloid sarcoma is an extramedullary tumor consisting of immature hematopoietic cells of granulocytic or monocytic differentiation. While rare, it can be seen in a variety of clinical settings and is most commonly associated with acute myeloid leukemia (AML), myelodysplastic syndromes (MDS) and myeloproliferative neoplasms (MPN). We present a rare case of myeloid sarcoma occurring in the bladder of a 56 year old male. Myeloid sarcoma may be difficult to recognize due to its rarity and clinical and morphologic similarity to many other conditions; however, swift diagnosis is necessary as it is considered equivalent to AML. Prognostic indicators for myeloid sarcoma have not been well established, but survival may be improved by undergoing chemotherapy designed to treat AML.