Cutaneous findings leading to a diagnosis of superior vena cava syndrome: A case report and review of the literature.

Superior vena cava syndrome, which occurs in approximately 15,000 persons in the United States annually, consists of a collection of symptoms and signs resulting from the obstruction of the superior vena cava (SVC). An early and prominent symptom of this condition is a constellation of superficial, dilated, vertically oriented and tortuous cutaneous venules or veins above the ribcage margins, often presenting as a blanchable violaceous eruption. Herein, we report a case of superior vena cava syndrome diagnosed in our dermatology clinic.

Chondroid syringoma: case report and review of the literature.

An 84-year-old man presented with an enlarging bluish, painless subcutaneous nodule on the glabella. The lesion had been excised 4 years prior and was diagnosed as chondroid syringoma, but had gradually regrown. The recurrent lesion was treated by surgical re-excision. Histopathological examination was again consistent with chondroid syringoma, and showed the following: 1) a chondroid matrix, 2) tubuloalveolar structures lined by a double epithelium, 3) ductal structures lined by a single epithelium, 4) nests of polygonal cells, and 5) the presence of keratinous cysts. Chondroid syringoma is a rare mixed tumor of the skin that was first described by Hirsch and Helwig. Characteristically, it is composed of a proliferation of epithelial cells set in a myxoid and chondroid matrix. Although chondroid syringomas are predominantly benign, malignant forms have been reported.

Pemphigus erythematosus associated with anti-DNA antibodies and multiple anti-ENA antibodies: a case report.

Pemphigus erythematosus (PE) is an autoimmune blistering disease combining features of pemphigus foliaceus (PF) and systemic lupus erythematosus (SLE). We report a case of PE associated with anti-double-stranded DNA (anti-dsDNA), anti-Smith (anti-Sm), anti-Ro (anti-SSA), and antiribonucleoprotein (anti-RNP) antibodies. This case required extensive immunosuppressive therapy. We treated our patient with a combination of systemic steroids, intramuscular gold injections, azathioprine, and hydroxychloroquine. The patient's response was complete remission--evaluated clinically, serologically, and immunohistochemically.

A masked NES in INI1/hSNF5 mediates hCRM1-dependent nuclear export: implications for tumorigenesis.

INI1 (integrase interactor 1)/hSNF5 is a component of the mammalian SWI/SNF complex and a tumor suppressor mutated in malignant rhabdoid tumors (MRT). We have identified a nuclear export signal (NES) in the highly conserved repeat 2 domain of INI1 that is unmasked upon deletion of a downstream sequence. Mutation of conserved hydrophobic residues within the NES, as well as leptomycin B treatment abrogated the nuclear export. Full-length INI1 specifically associated with hCRM1/exportin1 in vivo and in vitro. A mutant INI1 [INI1(1-319) delG950] found in MRT lacking the 66 C-terminal amino acids mislocalized to the cytoplasm. Full-length INI1 but not the INI1(1-319 delG950) mutant caused flat cell formation and cell cycle arrest in cell lines derived from MRT. Disruption of the NES in the delG950 mutant caused nuclear localization of the protein and restored its ability to cause cell cycle arrest. These observations demonstrate that INI1 has a masked NES that mediates regulated hCRM1/exportin1-dependent nuclear export and we propose that mutations that cause deregulated nuclear export of the protein could lead to tumorigenesis.

Heat transfer between two degrees of freedom.

A particularly simple chaotic nonequilibrium open system with two Cartesian degrees of freedom, characterized by two distinct temperatures T(x) and T(y), is introduced. The two temperatures are maintained by Nose-Hoover canonical-ensemble thermostats. Both the equilibrium (no net heat transfer) and nonequilibrium (dissipative) Lyapunov spectra are characterized for this simple system.