RESUMO
Pyoderma gangrenosum (PG) and erythema nodosum (EN) are rare skin conditions associated with inflammatory bowel disease (IBD), with increasing incidence as the disease progresses. We describe three cases of newly diagnosed IBD with cutaneous extraintestinal manifestations (EIMs) at the time of diagnosis. Three previously healthy patients presented with bloody diarrhea and concomitant nodular and ulcerating skin lesions at the onset of diarrhea. Dermatopathology showed PG and EN with endoscopic confirmation of ulcerative colitis. Clinical improvement was achieved with steroids and biological agents. These cases display the importance of a proper review of symptoms and a detailed workup of dermatological lesions prior to assuming infectious etiology.
RESUMO
BACKGROUND: The objective of this study was to identify prognostic factors for survival in patients with primary diffuse large B-cell lymphoma (DLBCL) of the adrenal gland. METHODS: Thirty one patients diagnosed with primary adrenal DLBCL from 14 Korean institutions and treated with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone) were analyzed. RESULTS: Complete remission (CR) and overall response rate after R-CHOP chemotherapy were 54.8% and 87.0%. The 2-year estimates of overall survival (OS) and progression-free survival (PFS) were 68.3% and 51.1%. In patients achieving CR, significant prolongations of OS (P = 0.029) and PFS (P = 0.005) were observed. Ann Arbor stage had no influence on OS. There was no significant difference in OS between patients with unilateral involvement of adrenal gland and those with bilateral involvement. When staging was modified to include bilateral adrenal involvement as one extranodal site, early stage (I or II) significantly correlated with longer OS (P = 0.021) and PFS (P <0.001). CONCLUSIONS: Contrary to prior reports, our data suggests that outcomes of primary adrenal DLBCL are encouraging using a regimen of R-CHOP, and that achieving CR after R-CHOP is predictive of survival. Likewise, our modified staging system may have prognostic value.