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.

A case of hyperpigmentation and acanthosis nigricans by testosterone injections.

Drug-related skin disorders may occur in many different ways. Despite pigmentary changes being less important for morbidity, these changes precipitate depressed mood and reduce self-confidence. Testosterone is a steroid hormone from the androgen group and primarily used for the treatment of hypogonadism in males. Testosterone replacement can cause skin problems like acne, hair loss, redness, pain, or infection at the injection site. The study was conducted on a 49-year-old man with adult onset idiopathic hypogonadotropic hypogonadism, which is an acquired form of isolated gonadotropin-releasing hormone deficiency. He was presented with lack of energy and decreased sexual function 10 years ago and was given an oil-based injectable blend of four esterized testosterone compounds as hormone replacement treatment in a urology polyclinic. He was referred to our polyclinic by endocrinologist because of progressive hyperpigmentation marked on his face and oral mucosa. In the present study, we report the first testosterone therapy-related facial and oral mucosal hyperpigmentation and acanthosis nigricans in the same patient.

Oral metronidazole treatment of lichen planus.

BACKGROUND: Oral metronidazole has been reported to be effective in some patients with idiopathic lichen planus (LP) who did not have concomitant protozoal infections of the intestinal or genital tracts. OBJECTIVE: Our purpose was to evaluate the efficacy of this drug in the treatment of generalized LP. METHODS: Nineteen patients with LP who were free from intestinal and genital protozoal infections were treated with oral metronidazole, 500 mg twice daily, for 20 to 60 days and were followed up for a period of 5 to 36 months. RESULTS: Fifteen patients (78.9%) improved with metronidazole treatment. Complete response was observed in 13 patients (7 women and 6 men). Two patients responded partially. Worsening of the lesions was observed in 1 of the 4 nonresponding patients. CONCLUSION: Metronidazole may be an alternative therapy for the treatment of generalized LP. Its immunomodulatory activity seems to be a possible mechanism of action besides its antimicrobial activity.

Interferon alfa-2a in the treatment of Behçet's disease.

BACKGROUND: There is no definite treatment for Behçet's disease. New drugs are being evaluated in cases unresponsive to conventional treatment modalities. OBJECTIVE: In this study we investigated the efficacy of interferon alfa-2a on skin lesions in 18 Behçet's disease patients who had predominantly mucocutaneous involvement. METHODS: Eighteen patients with Behçet's disease were treated with interferon alfa-2a at 3 million IU/day in the first week (three times a week), 6 million IU/day in the second week (three times a week), 9 million IU/day in the third week and thereafter (three times a week). Interferon alfa-2a was administered subcutaneously for a total of 12 weeks. RESULTS: At the end of the treatment the efficacy of the drug was found to be good in 9 patients and very good in 7 patients. Interferon alfa-2a is particularly effective in skin manifestations such as genital ulceration and erythema-nodosum-like lesions. It was also found to be effective in systemic manifestations such as fever, diarrhea and eye involvement. CONCLUSION: Our results indicate that interferon alfa-2a is a promising drug in the treatment of Behçet's disease.