RESUMO
Background and aim: Myeloproliferative disorders (MPD) (like polycythemia vera, essential thrombocythemia and primary myelofibrosis) are responsible for 50% cases of Budd-Chiari syndrome (BCS) and 35% cases of portal venous thrombosis (PVT) in western series. A point mutation at Val617Phe of Janus kinase 2 tyrosine kinase gene (JAK2V617F mutation) occurs in high proportion with MPD. This may be useful in diagnosing overt and latent form of MPD in intra-abdominal venous thrombosis (IAVT), consisting of BCS and PVT. Methods: In a 4 year prospective study from 2006 to 2009, JAK2 mutations were assessed in all patients diagnosed with MPD and IAVT attending our institution. Twenty three healthy individuals and 31 patients with non-MPD hematological disorders served as controls. All patients of idiopathic IAVT were tested for the mutation. Test for JAK2V617F mutation was carried out by allele specific polymerase chain reaction. Results: JAK2 V617F mutation was significantly more common in MPD patients (76%) than in non-MPD hematological disorders (0%) and healthy controls (0%). There was no statistical difference in presence of JAK2V617F mutation in patients of MPD with or without thrombosis (80% vs. 74%). In 58 patients with IAVT, the JAK2V617F mutation was present in 40%with BCS, 14% with PVT and 100% combined BCS+PVT). Conclusions: The JAK2V617F mutation occurs at high frequency in patients with MPD and IAVT. All idiopathic IAVT patients must be screened for JAK2V617F mutation to detect latent MPD. Detection of latent MPD by JAK2V617F mutation in BCS may change treatment strategy and outcome.
RESUMO
BACKGROUND: Psoriasis has different clinical variants, which mimic diverse dermatological conditions and may require a histopathological confirmation of the diagnosis. Studies to establish a clinicohistopathological concordance (and its determinants), in psoriasis and psoriasiform dermatitis are lacking. AIMS: The present study was designed (a) to correlate the clinicohistopathological features of psoriasis and psoriasiform dermatitis, and (b) to identify determinant(s) that may contribute to the diagnosis of psoriasis and psoriasiform dermatitis. METHODS: This was a prospective study involving 100 patients, with a single clinical diagnosis of psoriasis or with psoriasis as one of the differential diagnoses, and its correlation with histopathological features. RESULTS: The clinical features of typical scale (P = 0.0001) and Auspitz's sign (P = 0.0001), and histological evidence of suprapapillary thinning (P = 0.0001) and absent granular cell layer (P = 0.0001) were found to be statistically significant contributors to the clinicohistological concordance in cases of psoriasis. Vertical orientation of collagen bundles (P = 0.0001) and lymphocytic exocytosis (P = 0.003) were found to be significantly associated with diagnosis of psoriasiform dermatitis. CONCLUSION: The present study reconfirms the diagnostic accuracy of silvery white scale, Auspitz's sign, and Koebner's phenomenon in a clinical setting suggestive of psoriasis. However, in their absence, histological evidence of suprapapillary thinning and absent granular layer, in addition to the Munro microabscess and Kogoj's abscess, may contribute to the diagnosis of psoriasis. Similarly, vertical orientation of collagen bundles and lymphocytic exocytosis may point toward a diagnosis of psoriasiform dermatitis.
RESUMO
Herpes zoster or shingles is an acute vesico-bullous cutaneous infection characterized by dermatomal distribution, predominantly in adults. Extensive cutaneous dissemination has been reported in immunocompromised patients. However, its existence is documented in immunocompetent individuals as well. We report two children with disseminated herpes zoster, one of whom was immunocompromised secondary to severe mal-nutrition and had associated orbital septal cellulitis.