Effects of Behçet's disease on hearing thresholds and transient evoked otoacoustic emissions.

OBJECTIVES: The aim of this prospective study was to investigate hearing loss in patients with Behçet disease (BD). METHODS: The study group consisted of 20 patients (8 males, 12 females) with BD and 40 ears. The control group consisted of 20 healthy patients without BD (8 males, 12 females) and 40 ears. All subjects were evaluated by an otolaryngologic examination, pure-tone and high-frequency audiometry, and transient evoked otoacoustic emissions (TEOAEs). RESULTS: Sensorineural hearing loss (SNHL) was present in 25% of the ears on pure-tone audiometry and 60% of the ears on high-frequency audiometry in the study group. In the study group, at pure-tone and high-frequency audiometry, hearing thresholds were significantly higher than in the control group. For TEOAE values, in the BD group, each of the 1.0 to 4.0 kHz percent and amplitude values was significantly lower than in the control group. It was found that as age increased, hearing thresholds increased and TEOAEs decreased. As the disease duration got longer, hearing thresholds continued to increase and otoacoustic emissions at 3.0 kHz decreased. In males, hearing thresholds increased more whereas emissions decreased more than in females. CONCLUSION: Since SNHL is not infrequent in BD patients, patients may be evaluated regularly by pure-tone and high-frequency audiometry and TEOAEs. It may be possible to detect cochlear pathologies earlier than audiometric tests by TEOAEs.

Executive dysfunctions and depression in Behçet's disease without explicit neurological involvement.

This study aims to assess the executive functions and depression status in patients with Behçet's disease without explicit neurological involvement and to evaluate cognitive functions in this group of patients independent of accompanying depression. In the present study, 30 patients with Behçet's disease in the non-active phase of their illness and 30 healthy volunteers were included. In the evaluation of depression levels, Beck Depression Inventory was employed. The executive functions of the patients were evaluated by Wisconsin Card Sorting Test (WCST) and Stroop Test (ST). Beck Depression Inventory scores measured in the Behçet's disease group, in which no clinically serious depression was observed, were higher than the control group. Then, it was observed that the scores of neuropsychological tests of the Behçet's disease group were found to be significantly lower, especially in WCST, while significant relations were revealed between Beck Depression Inventory scores and WCST, ST scores. The authors' findings suggest that there may be factors other than depression causing executive impairment in patients with Behçet's disease without explicit neurological symptoms. Executive dysfunctions may occur in association with mild depressive states due to chronic disease stress or silent and/or future neurological involvement of Behçet's disease, especially in the frontal lobes.

A prospective randomized controlled trial to determine if cryotherapy can reduce the pain of patients with minor form of recurrent aphthous stomatitis.

BACKGROUND: Tissue cooling has long been used in the management of both acute and chronic pain. OBJECTIVE: To determine whether the application of cryotherapy can reduce the pain of patients with minor form of recurrent aphthous stomatitis. STUDY DESIGN: Twenty adult patients who had 2 discrete aphthous stomatitides in the labial mucosa at the same time were included in this prospective, randomized, and placebo-controlled study. One of the 2 aphthous stomatitides was treated with cryotherapy, the other serving as a control. The pain of aphthous stomatitis was scored by the patient on a 6-point scale (from 0 to 5). The size of the aphthous stomatitis was also measured. RESULTS: At any interval, no statistical difference was found between the cryotherapy-treated aphthous stomatitis and the control in the change in the value of pain severity, nor was any statistical difference found in the change in the size of the aphthous stomatitis. However, a trend toward less pain in the aphthous stomatitis receiving cryotherapy was noted. CONCLUSION: These results suggest that application of cryotherapy on minor form of recurrent oral aphthous stomatitis has no beneficial analgesic effect compared to placebo.

Mycosis fungoides and Sezary syndrome: therapeutic approach and outcome in 113 patients.

BACKGROUND: Mycosis fungoides (MF) and Sezary syndrome (SS) are the most common forms of cutaneous T-cell lymphoma (CTCL). Various topical and systemic therapeutic alternatives are available, but there is no standard or definite curative treatment regimen. When making a decision about the appropriate treatment modality, the age and compliance of the patient, stage of the disease, treatment accessibility, and previous treatment history should be considered. AIM: To determine the therapeutic response of patients with MF and SS to different treatment modalities. Patients were evaluated with respect to their clinical and demographic features. METHODS: One hundred and thirteen patients diagnosed clinically and dermatopathologically with MF and SS between March 1984 and June 2001 were included in the study. RESULTS: Of the 113 patients studied, 110 had a diagnosis of MF and three had a diagnosis of SS; 101 patients (89.4%) were diagnosed with early stage (IA, IB, IIA) and 12 (10.6%) with late stage (IIB, III, IVA, IVB) disease. The age at diagnosis varied between 12 and 81 years (mean, 45.6+/-15.8 years). Fifty-five (48.7%) patients were male and 58 (51.3%) were female. The duration of the skin lesions varied between 1.5 months and 32 years (mean, 6.1 years). Psoralen plus UVA (PUVA) was the most commonly used initial treatment modality in early stage disease (91%), with a complete remission (CR) rate of 80.4%. With PUVA+interferon-alpha (INF-alpha) treatment, CR was 57% in the early stages and 33.3% in the late stages. For late stage disease, systemic therapies, such as pentostatin, gemcitabine, and fludarabine, alone or in combination with INF-alpha, were preferred. Of the 113 patients, eight (7% of the total and 57.1% of the advanced stage cases) died of MF; 21.4% of the late stage patients showed partial remission and 14.2% showed CR. None of the patients diagnosed with early stage disease died of MF, but two (1.9%) progressed to late stage disease. CONCLUSIONS: PUVA and PUVA+INF-alpha are effective treatment modalities, especially for early stage MF. Once the disease has progressed, both MF and SS are very resistant to treatment regimens, including chemotherapeutic agents. It is important to diagnose and treat these diseases, especially MF, in the early stages for lasting remission.

Unilateral hyperhydrosis in Pourfour du Petit syndrome.

Upper limp hyperhydrosis is an idiopathic disease with bilateral involvement. However, Pourfour du Petit syndrome, the opposite of Horner syndrome, may result in unilateral upper limb hyperhydrosis. It occurs following hyperactivity of the sympathetic cervical chain as a consequence of irritation secondary to trauma. We report herein two cases with Pourfour du Petit syndrome showing unilateral upper limb hyperhydrosis. The patients presented with right-sided mydriasis and ipsilateral hemifacial hyperhydrosis. The onset of disease was followed by a trauma in both patients. They underwent upper thoracic sympathectomy with favorable outcome. A history of an antecedent trauma in patients with unilateral upper limb hyperhydrosis and anisocoria may imply a possible diagnosis of Pourfour du Petit syndrome.

Peripheral neuropathy in Behçet's disease.

Behçet's disease (BD) is a multisystem disorder. Since its first description, the involvement of many organ-systems has been studied. However, the involvement of the peripheric nervous system has not been well documented yet. Twenty-six patients involved in the study were without prominent complaints of neuropathic symptoms. They were surveyed through the outpatient clinic of the department of dermatology. Each patient filled out a total neuropathy score (TNS) questionnaire after their neurologic exam. A neurology specialist conducted the electrophysiological study. Our results were noteworthy because this complication was present without significant complaint. We hypothesized that the nerve dysfunction or peripheral neuropathy of BD is an axonal type of distal polyneuropathy and predominantly involves the lower extremities. We did not find a predominant motor or sensory nerve involvement. This complication is mentioned and reported rarely in BD.

Focal acral hyperkeratosis: a rare cutaneous disorder within the spectrum of Costa acrokeratoelastoidosis.

Acrokeratoelastoidosis and focal acral hyperkeratosis share similar clinical features and identical histologic epidermal alterations. These disorders are distinguished solely on the basis of the absence of elastorrhexis in the latter. We present a case of focal acral hyperkeratosis in a 9-year-old girl. The lesions consisted of translucent polygonal papules clustered on the thenar regions of the palms and over the metacarpophalangeal and interphalangeal joints. Histopathologic examination revealed orthohyperkeratosis within focal clavus-like depressions of the epidermis and prominent hypergranulosis. There was no evidence of alterations in elastic tissue. The clinicopathologic distinction between focal acral hyperkeratosis and acrokeratoelastoidosis is blurred. There is enough evidence to consider the former as a histologic variant of Costa acrokeratoelastoidosis syndrome, and a better nomenclature for this disorder would be "acrokeratoelastoidosis without elastorrhexis."

Reliability of Web-based teledermatology consultations.

We studied the reliability of teledermatology diagnoses made using a Web-based system. Clinical photographs and information relating to 125 patients were placed on a Web server. Three dermatologists made the most likely diagnosis via a Web interface. The reference diagnosis was made in a face-to-face consultation with a fourth dermatologist; where appropriate it was confirmed histologically. The teledermatologists were correct in 57% of cases when viewing the images alone. Their diagnostic accuracy improved to 70% when additional clinical information was available. The rate of agreement between the teledermatologists ranged from 44% to 70% (kappa= 0.22-0.32). Seventy-seven per cent of the patients were correctly diagnosed by at least two dermatologists when clinical information was provided. A Web-based system appears to be reliable for teledermatology. A single well trained teledermatologist may give better results than a group of less well trained clinicians.

BCL-2-related apoptosis markers in cutaneous human papillomavirus-associated lesions.

BACKGROUND: Human papilloma virus (HPV) is an etiological agent in benign and malignant epithelial tumors. Resistance to apoptotic stimuli by viral strategies represents an immunologic escape mechanism during virus-induced tumor development and is critical for efficient replication of the virus. OBJECTIVE: The aim of the present study was to investigate a role of bcl-family proteins in the anti-apoptotic pathways modulated by low-risk HPVs in the development of benign HPV-associated cutaneous tumors. METHODS: Forty lesional biopsy specimens from HPV-associated cutaneous lesions and 11 non-lesional control skin biopsies were studied by immunohistochemical analysis for the differential expressions of HPV antigens, the pro-apoptotic bax protein, and the anti-apoptotic bcl-2 and bcl-x proteins. RESULTS: Compared with the normal epidermis, bcl-2 and bcl-x expression were significantly reduced in the lesional epidermis. Bax was expressed in HPV-associated cutaneous lesions, although the expression did not reveal a significant deviation from that in normal skin. CONCLUSION: These findings indicate a discordant expression of bcl-2/ bcl-x and bax proteins in HPV-associated skin lesions and suggest that low-risk HPVs mediate other pathways that bypass the action of anti-apoptotic bcl-2 and bcl-x proteins. The presence of bax expression with a prominent decrease in bcl-2/ bax ratio and the lack of massive apoptosis in HPV-associated benign epithelial lesions may imply that interference with the pro-apoptotic proteins of bcl-family may constitute one of the several mechanisms mediated by HPV oncoproteins for the suppression of apoptotic process.

Examination of Bcl-2, Bcl-X and bax protein expression in psoriasis.

BACKGROUND: Psoriasis is an inflammatory skin disease characterized by epidermal hyperplasia and greatly accelerated epidermal turnover. The blockage of normal apoptotic process in the epidermis is one of the factors implicated in the pathogenesis of psoriasis. OBJECTIVE: The purpose of the present study was to elucidate whether bcl-family proteins are significantly involved in the hypothetical antiapoptotic cascade in lesional psoriatic epidermis. METHODS: Twenty-six lesional biopsy samples of 26 patients with psoriasis and five control specimens from normal skin were studied by immunohistochemical method for the differential expression of pro-apoptotic bax and antiapoptotic bcl-2 and bcl-x proteins. RESULTS: Compared with the normal epidermis, bcl-2 expression was significantly reduced, whereas bax and bcl-x were significantly overexpressed in the psoriatic epidermis. The localization of bcl-2/bax/bcl-x proteins in the psoriatic epidermis did not show a significant deviation from that in the normal epidermis. CONCLUSION: These findings indicate a discordant expression of bcl-2 and bax/bcl-x in psoriatic epidermis. Increased bcl-x expression might contribute to the antiapoptotic response in psoriatic keratinocytes. The presence of strong bax expression with a concomitant decrease in bcl-2 expression suggests either a functional defect in bax protein or an inherent/acquired resistance to bax-mediated apoptosis in psoriatic keratinocytes.

Juvenile subcorneal pustular dermatosis: a case report.

Subcorneal pustular dermatosis is a chronic, relapsing, pustular dermatosis that usually develops in elderly women. It is rarely seen in childhood and adolescence. The hallmark of the disease is formation of a subcorneal pustule detected on histopathologic examination. Herein we present a 13-year-old girl diagnosed with subcorneal pustular dermatosis based on the typical clinical and histologic findings. Direct and indirect immunofluorescence and serum protein electrophoresis had negative results. The patient partially recovered after 5 weeks of treatment consisting of oral dapsone and a topical steroid ointment.

HLA-class 1 and class 2 antigens in Turkish patients with pemphigus.

BACKGROUND: Pemphigus is an autoimmune disease which is more frequently seen in certain ethnic groups such as Jews. It is thought that exogenous factors may induce pemphigus in genetically predisposed individuals. Recent reports on HLA antigens indicate an increased frequency of HLA-class II antigens particularly HLA-DR4 among Jewish patients. Herein we investigated the antigen frequencies of HLA-A, B, C, HLA-DR and DQ in Turkish patients with pemphigus. METHODS: HLA class I and II antigens were typed by microdroplet lymphocyte cytotoxicity test in 33 patients with pemphigus and 100 healthy individuals. RESULTS: HLA-B35, B44, CW4, DR4, DR14, DQ8 and DQ4 antigens were significantly high in the study group whereas HLA-DR11, DQ7 and DQ2 antigens were high among the controls. The most striking differences were observed in HLA class II antigens. HLA DR14-DQ8 and HLA B35-DR14 haplotypes were the most frequently observed ones in the study group. CONCLUSIONS: We postulate that HLA-B35, B44, CW4, DR4, DR14, DQ4 and DQ8 antigens may be responsible for susceptibility to pemphigus while HLA-DR11, DQ7 and DQ2 antigens may have a protective role in the Turkish population.

Bcl-2-related proteins, alpha-smooth muscle actin and amyloid deposits in aggressive and non-aggressive basal cell carcinomas.

Aberrant expression of bcl-gene products has been implicated in the development of non-melanoma skin cancers. Recently, altered expression of alpha-smooth muscle actin has been proposed as predictive of tumour behaviour in basal cell carcinomas. The purpose of this study was to compare the aggressive and non-aggressive basal cell carcinomas in terms of bcl-gene products and alpha-smooth muscle actin expression. Fifty excisional biopsy samples were studied by immunohistochemical technique for the differential expressions of bcl-2, bax, bcl-x and alpha-smooth muscle actin. Bcl-2, bcl-x and bax were expressed in 34 (68%), 38 (76%) and 41(82%) specimens, respectively. Immunoreactivity for alpha-smooth muscle actin was noted both in tumour nests (64%) and within the stroma (54%). There was a significant difference between aggressive and non-aggressive basal cell carcinomas in terms of bcl-2 and stromal alpha-smooth muscle actin immunoreactivity. Non-aggressive basal cell carcinomas display a concordant expression of bcl-family proteins, whereas aggressive tumours reveal a discordant pattern. An increased expression of stromal alpha-smooth muscle actin with a concomitant decrease or loss of bcl-2 expression may be highly suggestive of aggressiveness in basal cell carcinoma.