Belkız Uyar

Evaluation of Arterial Stiffness in Patients with Behçet's Disease by Using Noninvasive Radiological Methods such as Intima-Media Thickness of the Carotid, Ankle-Brachial Pressure Index, Coronary Artery Calcium Scoring, and Their Relation to Serum Fetuin-A Levels: A Case-Control Study

Background Behçets disease (BD) is a chronic, recurrent inflammatory systemic vasculitis. Evidence for increased atherosclerosis in BD has been observed. The relation between cardiovascular risk factors and increased atherosclerosis in patients with BD is still controversial. Objective We performed this study to evaluate arterial stiffness in patients with BD by using noninvasive radiological methods such as carotid artery intima-media thickness (CIMT), ankle-brachial pressure index (ABPI), coronary artery calcium score (CACaS), and their relation to serum fetuin-A levels, which was recently found to be important in vascular calcification. Methods This prospective study included 26 patients with BD and 25 control subjects. In all patients, the CIMT, ABPI, CACaS, and serum fetuin-A levels were examined. Results The CIMT and CACaS were statistically higher and the ABPI was statistically lower in BD patients than in the control group. All p-values were <0.001. Positive correlations were found between the CACaS and CIMT, and negative correlations were found between the CACaS and ABPI. Although the values of fetuin-A were higher in BD, the difference was not statistically significant (p=0.064). However, the correlations found between fetuin-A levels and CIMT and between fetuin-A levels and CACaS were significant. Conclusion The CIMT, CACaS, and ABPI are all useful in detecting structural and functional vascular damage in BD.

Aquagenic syringeal acrokeratoderma.

Aquagenic syringeal acrokeratoderma is a rare, transient, and usually bilaterally symmetric, palmoplantar keratoderma. Patients complain of tingling and pain in the hands starting a few minutes after exposure to water and lasting for 20-30 minutes after removal. Clinically, there is marked wrinkling with edematous white papules on the palms or, less often, the soles. We present the case of a 21-year-old woman who used spironolactone for polycystic ovary syndrome and had similar clinical features 2 weeks later, after withdrawing the drug.

A 17-year-old with neurofibromatosis and spontaneous coronary artery dissection.

A 17-year-old girl with neurofibromatosis type 1 presented with unstable angina. Cardiac catheterization revealed an aneurysm with thrombus in the left anterior descending coronary artery. She was discharged on medical treatment but returned 2 months later with severe chest pains. Angiography revealed an increase in the size of the aneurysm in the left anterior descending coronary artery, with thrombus and dissection. The patient underwent coronary artery bypass surgery. Follow-up after 1 year revealed no problems.

Giant chondroid syringoma radiologically mimicking malignancy

Chondroid syringoma, or mixed tumor of skin, is a relatively rare, usually benign sweat gland tumor, most often seen in the head-and-neck region. Rare malignant examples have been reported, commonly involving the extremities. We report here a case radiologically mimicking a malignant neoplasm, but histologically-proven benign subcutaneous chondroid syringoma, arising in the anterior aspect of the upper thigh of a 59-year-old male.

Effects of the 755-nm Alexandrite laser on fine dark facial hair: Review of 90 cases

Laser hair removal is a relatively effective method for thick hair. Despite the risk for induction of fine hair growth, application of laser for fine dark hair is sometimes inevitable. We investigate the effects of 755‐nm Alexandrite laser on fine dark facial hair and evaluate the induction rates of fine hair growth and case satisfaction. In the present study, the thickening rate of hairs (33.33%) was found to be higher than the previously published rates. However, reduction of hair density can be obtained when the laser sessions are continued.

Effects of isotretinoin on the thyroid gland and thyroid function tests in acne patients: A preliminary study

Background: Isotretinoin is widely used in the treatment of acne. Aims: We investigated the effects of isotretinoin on thyroid function tests and thyroid volume in acne patients. Methods: In this prospective study, a total of 104 acne patients were included. Sixty-six patients were treated with isotretinoin for at least 4 months. Thirty eight patients were included in the control group. The levels of thyroid stimulating hormone, free triiodothyronine, free thyroxine, antithyroglobulin and antithyroid peroxidase antibodies were measured and a thyroid ultrasound was performed in all the subjects before treatment and 4 months after treatment. A “p” value of < 0.05 was considered significant. Results: In the isotretinoin-treated group, thyroid stimulating hormone levels increased significantly during isotretinoin treatment (P = 0.018). Free triiodothyronine, free thyroxine, anti-thyroid peroxidase levels and thyroid volume decreased significantly during treatment (P = 0.016, P= 0.012, P= 0.006, P = 0.020 respectively). Limitations: The major limitation of this study is the lack of follow-up data after the cessation of isotretinoin therapy in acne patients. Conclusion: Patients treated with isotretinoin should be monitored with thyroid function tests.

Intracardiac multiple thrombus formation as a rare manifestation of primary antiphospholipid antibody syndrome: a case report

Sir, Antiphospholipid syndrome (APS) has been described by Graham R. Hughes as a combination of the clinical symptoms of arterial and venous thromboembolism, with the presence of autoantibodies. Other features of APS include recurrent spontaneous abortion, thrombocytopenia, chorea, migraine, cutaneous symptoms, and valvular heart disease. Antiphospholipid antibodies may be associated with some connective tissue diseases, such as systemic lupus erythematosus, as well as some infectious diseases. The disease may accompany HIV, hepatitis C, syphilis, and other infections, including cytomegalovirus. APS can sometimes be detected in healthy patients. In the absence of an underlying disorder, the syndrome is classified as primary. The following report details the case of a patient with intracardiac thrombus formation as a first manifestation of primary antiphospholipid antibody syndrome. A 25-year-old man was admitted to our cardiovascular surgery department with dyspnea of increasing severity, exhaustion, cognitive disturbance and vertigo. Three days before the hospital admission, he suffered shortness of breath and palpitation, and sought attention in the emergency room for further evaluation. Cardiac auscultation revealed a third heart sound (gallop rhythm) and grade III/VI systolic murmur on the second–third intercostal space over the left sternal border. Transthoracic and transoesophageal echocardiography revealed a mobile mass measuring 32 25 25mm, adhered to both leaflets of the mitral valve (Figure 1(a) and (b)). The mass was closely attached to the free edge of the mitral leaflets. Myxoma and bacterial/nonbacterial vegetation were considered in the differential diagnosis of the mass. Following the decision of the our hospital’s council of cardiology and cardiovascular surgery, the patient went to the operation room with a cardiopulmonary bypass to undergo surgical removal of the mass, due to progressive dyspnea and possible impending fatal embolism. During surgery, a 3 3 2 cm wellorganized, thrombus-like mass adherent to the left atrial surface of the both leaflets of the mitral valve became visible. The mass was friable and close to the leaflets’ free edge. The surgical team easily separated the mass from the leaflet, and completely removed it (Figure 2). The surgical specimen underwent histopathological examination. Intraoperative examination revealed that aortic, pulmonary, and tricuspid valve apparatus were not affected and normal. The histopathology reported blood fibrin, mesothelial cells, organized necrotic thrombus formation, or vegetation-like lesions. His other system questioning was normal. Anti-cardiolipin IgM was at 20.2U/ ml (normal 12); anti-cardiolipin IgG at 120U/ ml (normal 12); anti-b2-glycoprotein-1 at 200U/ml (normal 20); lupus anticoagulant at 89.4 sn (normal 31–44). According to international classification criteria, the patient was diagnosed with primary APS because of the suspected intracardiac thrombosis, marked by prolonged activated partial thromboplastin time and elevated anti-cardiolipin antibody titers. His general condition was good following surgery. Postoperative recovery was uneventful, and he was discharged in good condition with anticoagulation treatment. In conclusion, although intracardiac mass caused by APS is uncommon, it should be kept in mind while searching for an accurate diagnosis. Early removal of the mass and maintenance anticoagulation therapy are essential for the prevention of undesired catastrophic events.

Multiple pilar sheath acanthomas on the scrotal region

Turkderm-Turk Arch Dermatol Venereolgy 2017;51:18-20

Sweet's syndrome exhibiting the Koebner phenomenon at the site of dermatofibroma

Sweet′s syndrome (SS), also known as acute febrile neutrophilic dermatosis, is characterized by fever, neutrophilia, and inflammatory skin lesions. SS can be associated with several conditions, such as infections, malignancy, autoimmune disease, vaccination, pregnancy, and drug exposure. We present a woman with SS; skin lesions developed at the site of dermatofibroma and scratched area. To the best of our knowledge, this is the first case of SS developed at the site of dermatofibroma.

Basaloid follicular hamartoma on the upper eyelid

Basaloid follicular hamartoma (BFH) is a benign rare neoplasm of the hair follicles whose clinical and histological appearance is very similar to basal cell carcinoma. Although these hamartomas are considered to be benign lesions, malignant differentiations have been reported. It may be generalized or localized, familial or sporadic, and BFH can be accompanied by systemic diseases. Although there are many clinical forms of BFH, they all have the same histopathological features. Basaloid follicular hamartoma is a folliculocentric tumor limited to the superficial dermis. Involvement of the deep reticular dermis or soft tissue is not seen in BFH [1]. We present a 52-year-old man with a solitary, hyperpigmented, asymptomatic, slow growing skin tumor on his left upper eyelid. A 52-year-old man presented with a slowly developing asymptomatic left upper eyelid lesion (over 4 years). Dermatological examination showed a solitary, smooth surfaced, hyperpigmented nodule measuring 1 cm in diameter (Figure 1), and there were no other similar skin lesions or significant internal diseases exhibited. He had no family history of similar lesions. The lesion was locally excised, and the specimen was grossly measured to be 1.2 × 0.7 × 0.2 cm. Figure 1 A solitary, smooth surfaced, hyperpigmented 1 cm nodule is located on the left upper eyelid of a 52-year-old man Low-power light microscopy revealed a well-circumscribed and completely removed lesion in the dermis, without connections to the epidermis (Figure 2). Microscopically, the tumor revealed strands and cords of small basaloid cells emanating from the infundibular portion of the hair follicle. The tumor stroma was scant and mildly fibrocellular. There was no nuclear pleomorphism, mitotic activity, apoptotic cells, or cleft formation between the tumor and the stroma (Figure 3), and upon immunohistochemical examination, Bcl-2 stained only in the outermost basal cells (Figure 4). Cd34 was positively stained in the peritumoral stroma and blood vessels (Figure 5), and CD10 was stained in the peritumoral stroma as well as the matrical cells (Figure 6). Figure 2 Low-power light microscopy revealed a well-circumscribed and completely removed lesion in the dermis, without connections to the epidermis (hematoxylin-eosin, original magnification at 40×) Figure 3 A biopsy specimen with basaloid follicular hamartoma shows strands and cords of small, basaloid cells emanating from the infundibular portion of the hair follicle. The tumor stroma was scant and mildly fibrocellular. There was no nuclear pleomorphism, ... Figure 4 Bcl-2 stains only the outermost basal cells in BFH (Bcl-2, 100×) Figure 5 CD34 is positive in the peritumoral stroma and blood vessels (CD34 100×) Figure 6 CD10 stains the peritumoral stroma of BFH, as well as the matrical cells (CD10 100×) Basaloid follicular hamartoma was first described in 1969 by Brown et al. as “generalized hair follicle hamartoma” with associated alopecia, aminoaciduria, and myasthenia gravis [2]. The term “basaloid follicular hamartoma” was first used for a patient who had a localized and solitary type of the lesion, without associated abnormalities, by Mehregan and Baker in 1985 [3]. Morohashi et al. described BFH as an abortive growth of secondary hair germs with a limited differentiation toward the upper follicular portion of the hair shaft [4]. Basaloid follicular hamartoma may manifest with different clinical presentations, such as a solitary lesion, or as multiple lesions with a generalized or localized distribution. Basaloid follicular hamartoma may present as individual or linearly distributed, small, skin-colored to brown papules or plaques, or as multiple lesions in a generalized distribution on the face, scalp, and occasionally, the trunk. Basaloid follicular hamartoma may be a familial, congenital, or acquired condition. Several forms of generalized BFH have been described: (1) sporadic form, multiple BFH without systemic disease; (2) generalized acquired form, female patients with generalized BFHs associated with alopecia and autoimmune diseases, such as myasthenia gravis or systemic lupus erythematosus, in which the lesions are found mainly on the face and periorificial areas; (3) generalized familial form, an autosomal dominant disease that may or may not be associated with hypotrichosis, hypohidrosis, and palmoplantar pitting, which appears on the face and genital region; (4) generalized congenital form, generalized BFH associated with other ectodermal defects, such as hypotrichosis and punctate keratotic pits, on the palms and soles and with cystic fibrosis [1]. The localized forms of BFH present as linear unilateral lesions or as plaques with alopecia [3–6]. The linear unilateral type of BFH is associated with lines of Blaschko and presents at birth or appears in early childhood [3, 5, 6]. Solitary BFH was first described in 1992 as a smooth plaque or a papule appearing most commonly on the face or scalp [7]. The pathogenesis of BFH has been linked to a mutation in the PTCH (patched) gene on chromosome band 9q23. However, this mutation is thought to be less severe than the PTCH gene mutation demonstrated in nevoid basal cell carcinoma syndrome (NBCS) [8–10]. The clinical differential diagnosis for BFH depends on its presentation. The most common misdiagnoses for individual lesions include basal carcinoma (BCC), intradermal melanocytic nevus, seborrheic keratosis, sebaceous hyperplasia, syringoma, angiofibroma, trichilemmoma, steatocystoma, trichoepithelioma (TE), basal cell hamartoma with follicular differentiation, and hamartoma of the sebaceous follicles. When presenting as a plaque, nevus sebaceous, lupus erythematosus, and sarcoidosis should be considered. Basaloid follicular hamartoma in a linear distribution may mimic linear epidermal nevus, lichen striatus, linear morphea, and basal cell nevus. The differential diagnosis of generalized BFH could include generalized follicular hamartoma syndrome, tuberous sclerosis, Cowden disease, multiple trichoepitheliomas, nevoid basal cell nevus syndrome, Rombo syndrome, and multiple tumors of the follicular infundibulum [11]. Basaloid follicular hamartoma is often misdiagnosed as trichoepithelioma or basal cell carcinoma, histopathologically [1]. Specifically, BFH consists of malformed and distorted hair follicles composed of cords and strands of basaloid cells. These cells are arranged in a radial, anastomosing fashion and may arise from the follicles and/or show an epidermal attachment. The tumor cells are bland, without nuclear pleomorphism, and mitotic activity is rare or absent, with little to no single cell necrosis. While the presence of peripheral palisading has been reported, this feature is typically lacking to the degree seen in BCC. The stroma is scant or absent, and when present, consists of eosinophilic compact collagen with no fibrocytes. Clefts within the fibrous stroma have been reported, and minimal clefting between the tumor and stroma has been observed, but it is not a well-accepted feature of BFH. Mucinous ground substance, if present, is usually subtle; furthermore, BCC displays a variety of histological patterns. The neoplastic cells may involve and destroy preexisting hair follicles and the interfollicular dermis, and sometimes, infiltrate the deeper dermis, subcutaneous fat, and skeletal muscle [12]. Differentiating BFH from BCC can be the most difficult aspect of the diagnosis, and additional immunohistochemical stains may help to separate BFH from BCC. Ki-67 (a proliferative marker associated with mitosis) and Bcl-2 staining are more prominent in BCC than in BFH. Bcl-2 stains only the outermost basal cells in BFH. CD 34 is an intercellular adhesion protein and cell surface glycoprotein; the ligand is CD62L (L-selectin). It is also called hematopoietic progenitor cell antigen and is a commonly used marker of hematopoietic progenitor cells and endothelial cells. CD34 also stains stromal cells and dendritic cells. In differential diagnosis it is important that the stromal cells of the BFH stain positive for CD34, but the stromal cells of BCC are negative [1, 12]. Although in BCC the stromal and tumor cells stain with CD10, in BFH, the peritumoral stroma and matrical cells stain with CD10 [13]. Histologically, TE has distinct islands of basaloid cells in a lacelike or adenoidal network and, occasionally, as solid aggregates; additionally, they exhibit a more nodular growth pattern than BFH. The tumor islands show peripheral palisading as in BCC; however, the stroma lacks the retraction artifact seen in BCC. In TE, the fibrocystic stroma is more prominent than in BFH, and it predominates over the epithelial portion. Additionally, in TE, normal follicular bulbs and papillae are seen [11]. While both BFH and TE have keratin cysts consisting of a fully keratinized center, surrounded by basophilic cells without high-grade atypia and mitoses, they are more prominent in TE. We report a case of solitary BFH that developed on the left upper eyelid of a man. Since only a few cases have been reported, presenting case reports will increase awareness of this disorder. Although BFH is a benign rare neoplasm of the hair follicles, it is important to differentiate it due to the malignancy risk and similar characteristics to other benign tumors.