Ear, nose and throat involvement in patients with pemphigus vulgaris: correlation with severity, phenotype and disease activity.

BACKGROUND: Pemphigus vulgaris (PV) is the most common clinical form of pemphigus that is characterized by easily ruptured or loose bulla formation on skin and/or mucosa. The frequency of the ear, nose and throat involvement of PV is not clearly defined. OBJECTIVES: To evaluate the frequency of ear, nose and throat (ENT) involvement in patients suffering from PV who had been recently diagnosed or exacerbated under follow up and to determine the association with ENT symptoms, clinical involvement, severity and duration of pemphigus vulgaris. MATERIALS AND METHODS: The study group included a total of 38 PV patients comprising 24 new patients and 14 patients who showed exacerbations while on complete remission or under treatment. All patients were asked about ENT symptoms and endoscopic examination were performed to evaluate the presence of nasal, pharynx, larynx and ear involvement. RESULTS: Of the 38 patients, 33 (87%) had active PV lesions on endoscopic evaluation. Twenty-five patients (66%) had lesions on pharynx, twenty-one (55%) on larynx, twenty-nine (76%) on nasal mucosa and four (10%) on the ear mucosa. ENT involvement was not associated with the severity and the clinical involvement of the disease. Pharyngeal and nasal involvement was significantly associated with symptoms, while laryngeal and ear involvement was not found to be significantly associated with symptoms. Nasal and ear involvement was not associated with the duration of the disease while pharyngeal and laryngeal involvement favored newly diagnosed patients. CONCLUSIONS: Our results revealed that high number of patients with PV may present with active ENT lesions, furthermore patients with ear, nose and throat involvement may be asymptomatic and active lesions may be found in patients without any ENT symptoms. Therefore it should be considered that to understand the real extent of PV involvement, endoscopic ENT evaluation should be performed in patients with or without symptoms.

Microalbuminuria as an early marker of renal involvement in Behcet's disease: it is associated with neurological involvement and duration of the disease.

BACKGROUND: Despite its nature as a systemic vasculitis, renal involvement is known to occur infrequently in Behçet's Disease (BD). OBJECTIVES: Our aim was to investigate proteinuria, microhematuria and microalbuminuria in 24-h urine and evaluate subclinical or symptomatic renal involvement in BD patients. METHODS: Two hundred and eleven patients who fulfilled the International Behçet's Disease criteria were included in the study. After urine analysis, five of 12 patients who were found to have proteinuria underwent renal biopsy, while 199 patients without proteinuria were investigated for microalbuminuria (MA). RESULTS: A total of 34 (16.1%) patients were found to have renal involvement including 22 (11.1%) with MA and 12 with proteinuria (5.6%). Renal biopsies resulted as focal glomerulosclerosis in three, membranous glomerulosclerosis in one and secondary amyloidosis in two patients. Neurological involvement was found to be significantly more prevalent in patients with MA (P < 0.01). Neurological involvement and duration of disease (> or = 10 years) was found to increase the risk for MA by 21.75-fold and 5.03-fold, respectively. Though age over 40 years, thrombophlebitis, HLA B51 haplotype and ophthalmological involvement were not found to be significantly associated with MA; these parameters increased the risk for MA. CONCLUSIONS: Renal involvement may be more prevalent in BD than it has been recognized; it usually presents with asymptomatic microhematuria, proteinuria and/or microalbuminuria; therefore clinicians must check 24-h urine for the presence of proteinuria, microhematuria and microalbuminuria; especially in patients who are aged over 40 years, have a longer duration of the disease and multisystem involvement.