Atypical Varicella-Zoster Kaposi Varicelliform Eruption in Sézary Syndrome.

Patients with mycosis fungoides experience considerable morbidity and mortality from secondary bacterial and viral infections. Staphylococcus aureus, ß-hemolytic streptococci, herpes simplex virus, and herpes zoster virus remain the most common infectious pathogens in this group of patients. With depressed cellular immunity and diminished skin barrier as the main precipitating risk factors, immunocompromised patients can often present with an atypical presentation of a common dermatologic condition. The case herein discusses a clinically atypical nonvesicular Kaposi varicelliform eruption secondary to a varicella-zoster virus in a patient with Sézary syndrome. Concurrent polypharmacy in these patients is also a risk factor for development of drug hypersensitivity reactions. However, given their immunocompromised status, first and foremost, a careful inspection should be made of every atypical skin eruption in search of an infectious etiology, and afterward, an appropriate treatment should be promptly initiated.

Inhibition of type 4 cyclic nucleotide phosphodiesterase blocks intracellular TLR signaling in chronic lymphocytic leukemia and normal hematopoietic cells.

A subset of chronic lymphocytic leukemia (CLL) BCRs interacts with Ags expressed on apoptotic cells, suggesting that CLL BCRs have the potential to internalize apoptotic cell RNA- or DNA-containing fragments with resultant activation of TLR7 or TLR9, respectively. By blocking cAMP degradation, type 4 cAMP phosphodiesterase (PDE4) inhibitors activate cAMP-mediated signaling and induce apoptosis in CLL cells. In this study, we show that autologous irradiated leukemic cells induce proliferation in CLL cells and that such proliferation is blocked by a TLR7/8/9 inhibitor, by DNase, and by the PDE4 inhibitor rolipram. Rolipram also inhibited CLL cell proliferation induced by synthetic TLR7 and TLR9 agonists, as well as TLR agonist-induced costimulatory molecule expression and TNF-a (but not IL-6 or IL-10) production. Whereas treatment with a TLR9 agonist protected IgH V region unmutated, but not mutated, CLL cells from apoptosis, PDE4 inhibitors augmented apoptosis in both subtypes, suggesting that cAMP-mediated signaling may abrogate a TLR9-mediated survival signal in prognostically unfavorable IGHV unmutated CLL cells. Rolipram inhibited both TLR7/8- and TLR9-induced IFN regulatory factor 5 and NF-kB p65 nuclear translocation. PDE4 inhibitors also blocked TLR signaling in normal human immune cells. In PBMC and CD14-positive monocytes, PDE4 inhibitors blocked IFN-a or TNF-a (but not IL-6) production, respectively, following stimulation with synthetic TLR agonists or RNA-containing immune complexes. These results suggest that PDE4 inhibitors may be of clinical utility in CLL or autoimmune diseases that are driven by TLR-mediated signaling.

Spontaneous intraperitoneal hemorrhage as the initial presentation of a gastrointestinal stromal tumor: a case report.

BACKGROUND: Spontaneous hemoperitoneum is rare. The most common etiologies are gynecologic, splenic, and hepatic. Gastrointestinal stromal tumors (GISTs) are commonly associated with intraluminal bleeding, but rarely with spontaneous hemoperitoneum. We report a case of spontaneous hemoperitoneum caused by a gastric GIST. CASE REPORT: A 54-year-old male presented with the acute onset of abdominal pain and a drop in hemoglobin. Subsequent evaluation, including a CT, MRI, and EUS, revealed a 1.2-cm mass along the greater curvature of the stomach and associated hemoperitoneum. The patient was taken electively to the operating room for laparoscopic removal of the mass. Pathology confirmed that it was a GIST. CONCLUSION: GIST is a rare clinical entity that infrequently presents with spontaneous hemoperitoneum. Emergent treatment should be guided towards treating the spontaneous hemoperitoneum.