Deniz Yucelten

Comparison of finasteride versus spironolactone in the treatment of idiopathic hirsutism

Abstract Objective: To compare the efficacy of finasteride and spironolactone in the treatment of idiopathic hirsutism. Design: Prospective, randomized, single-blind study. Setting: A tertiary hirsutism clinic. Patient(s): Forty women with idiopathic hirsutism were selected. Intervention(s): Patients were assigned randomly to receive either 5 mg of finasteride or 100 mg of spironolactone for 9 months. Main Outcome Measure(s): Hirsutism scores were measured according to the Ferriman-Gallwey scoring system, and side effects were monitored for 9 months of treatment. Blood samples were taken at each visit for assessment of endocrine, biochemical, and hematologic parameters. Result(s): Hirsutism scores were decreased significantly in both groups at the end of 9 months. The mean percent change (±SD) in hirsutism scores in the finasteride and spironolactone groups was as follows: 5.91% ± 7.18% and 20.60% ± 12.59% at 3 months, 10.61% ± 12.18% and 32.57% ± 15.68% at 6 months, and 15.15% ± 15.38% and 42.36% ± 12.31% at 9 months, respectively. There was a significantly better response with spironolactone treatment at the end of 9 months. Eleven (55%) of 20 patients in the spironolactone group experienced side effects. However, none of them stopped treatment because of side effects. Conclusion(s): The present data suggest that both finasteride and spironolactone are effective in the treatment of idiopathic hirsutism. However, it appears that the spironolactone group responded significantly better.

Chemotherapy-induced acral erythema in leukemic patients: a report of 15 cases.

Background Chemotherapy‐induced acral erythema is a distinct localized cutaneous response to certain systemic chemotherapeutic agents.

Comparison of spironolactone-oral contraceptive versus cyproterone acetate-estrogen regimens in the treatment of hirsutism.

OBJECTIVE To compare the efficacy of two antiandrogens, cyproterone acetate (CPA) and spironolactone, in the treatment of hirsutism. DESIGN Prospective randomized single-blinded study. SETTING A tertiary hirsutism clinic. PATIENTS Forty-two premenopausal patients with hirsutism were selected. INTERVENTIONS Subjects were randomized to receive either 100 mg spironolactone and an oral contraceptive (OC) containing 150 microg desogestrel and 30 microg ethinyl E2 or 50 mg CPA daily on days 1 to 10 of the menstrual cycle, which was administered with 35 microg ethinyl E2 daily on days 1 to 21. MAIN OUTCOME MEASURES Hirsutism scores were measured according to Ferriman-Gallwey scoring system and side effects were monitored for 9 months of treatment. Blood samples were taken at each visit for assessment of endocrine, biochemical, and hematologic parameters. RESULTS Hirsutism scores were decreaded significantly in both groups at the end of 9 months. The percent of change in hirsutism scores in CPA and spironolactone group were as follows: 19.23% +/- 14.77% and 24.48% +/- 14.27% at 3 months; 39.01% +/- 19.77% and 37.46% +/- 16.90% at 6 months; and 51.89% +/- 20.87% and 46.39% +/- 16.10% at 9 months, respectively. There was a trend toward a better response with CPA treatment, which did not achieve significance. None of the patients stopped treatment because of side effects. CONCLUSION The present data suggest that both spironolactone and CPA were similarly effective in treatment of hirsutism.

Novel C16orf57 mutations in patients with Poikiloderma with Neutropenia: bioinformatic analysis of the protein and predicted effects of all reported mutations

BackgroundPoikiloderma with Neutropenia (PN) is a rare autosomal recessive genodermatosis caused by C16orf57 mutations. To date 17 mutations have been identified in 31 PN patients.ResultsWe characterize six PN patients expanding the clinical phenotype of the syndrome and the mutational repertoire of the gene. We detect the two novel C16orf57 mutations, c.232C>T and c.265+2T>G, as well as the already reported c.179delC, c.531delA and c.693+1G>T mutations. cDNA analysis evidences the presence of aberrant transcripts, and bioinformatic prediction of C16orf57 protein structure gauges the mutations effects on the folded protein chain.Computational analysis of the C16orf57 protein shows two conserved H-X-S/T-X tetrapeptide motifs marking the active site of a two-fold pseudosymmetric structure recalling the 2H phosphoesterase superfamily. Based on this model C16orf57 is likely a 2H-active site enzyme functioning in RNA processing, as a presumptive RNA ligase.According to bioinformatic prediction, all known C16orf57 mutations, including the novel mutations herein described, impair the protein structure by either removing one or both tetrapeptide motifs or by destroying the symmetry of the native folding.Finally, we analyse the geographical distribution of the recurrent mutations that depicts clusters featuring a founder effect.ConclusionsIn cohorts of patients clinically affected by genodermatoses with overlapping symptoms, the molecular screening of C16orf57 gene seems the proper way to address the correct diagnosis of PN, enabling the syndrome-specific oncosurveillance.The bioinformatic prediction of the C16orf57 protein structure denotes a very basic enzymatic function consistent with a housekeeping function. Detection of aberrant transcripts, also in cells from PN patients carrying early truncated mutations, suggests they might be translatable. Tissue-specific sensitivity to the lack of functionally correct protein accounts for the main cutaneous and haematological clinical signs of PN patients.

Recurrence rate of hirsutism after 3 different antiandrogen therapies

BACKGROUND Although antiandrogens are frequently and successfully used to treat hirsutism, little attention has been paid to optimal duration of treatment and recurrence rate after cessation of therapy. OBJECTIVE Our purpose was to determine the recurrence rate of hirsutism after 3 different antiandrogen therapies. METHODS Eighty-one hirsute women referred to a tertiary hirsutism clinic were assigned to one of three regimens: spironolactone 100 mg/day with an oral contraceptive, cyproterone acetate 50 mg/day on days 1 to 10 with an oral contraceptive, or flutamide 250 mg twice a day. Hirsutism scores according to the Ferriman-Gallwey scoring system and endocrine parameters were evaluated before, during, and 1 year after withdrawal of treatment regimens. RESULTS Hirsutism scores decreased significantly and similarly in spironolactone, flutamide, and cyproterone acetate treatment groups. However, 1 year after withdrawal of treatment in all antiandrogen therapy groups, hirsutism returned. CONCLUSION Antiandrogens are effective in the treatment of hirsutism. However, cessation of antiandrogen therapy is followed by recurrence.

Experience with intravenous immunoglobulin in severe childhood atopic dermatitis

6. Shiohara T, Inaoka M, Kano Y. Drug-induced hypersensitivity syndrome (DIHS): a reaction induced by a complex interplay among herpesviruses and antiviral and antidrug immune responses. Allergol Int. 2006;55:1--8. 7. Picard D, Janela B, Descamps V, D’Incan M, Courville P, Jacquot S, et al. Drug reaction with eosinophilia and systemic symptoms (DRESS): a multiorgan antiviral T cell response. Sci Transl Med. 2010;2, 46ra62.

Intravascular/Intralymphatic Histiocytosis: A Report of 3 Cases.

Intravascular/intralymphatic histiocytosis (IV/ILH) is a rare, reactive cutaneous condition, with uncertain pathogenesis. It may be associated with various inflammatory and neoplastic diseases. Although the clinical presentation is various, the biopsies reveal dilated vessels, mostly lymphatics, containing aggregates of histiocytes within their lumina. We described 3 cases of IV/ILH with different clinical presentations. In the first case, the patient presented with lymphedema in the genital region without any underlying disease. However, the second and third cases had reticular erythematous skin lesions. The second case had common variable immunodeficiency disease, rheumatoid arthritis, inflammatory bowel disease, and a history of a lymphoproliferative lesion. The third case had metal prostheses at both his right and left knees. In all these 3 cases, histopathologic and immunohistochemical findings were similar to each other and to those cases reported in the literature. In addition, the third case was admixed with reactive angioendotheliomatosis. In the second case, the endothelium of the ectatic vessels expressed CD31 and CD34, but not D2-40/podoplanin, pointing out that these vessels were blood vessels rather than lymphatics, differing from the other 2 cases. In conclusion, we believe, the most convincing statement about IV/ILH is that it is not a distinct clinicopathologic entity, but a histopathologic feature found as a part of a constellation of inflammatory changes or many other conditions.

Vulvar cicatricial pemphigoid in a child

Cicatricial pemphigoid is a rare disease primarily affecting the elderly. Only a few cases of childhood cicatricial pemphigoid have been reported in the literature. We present a case of cicatricial pemphigoid involving the vulvar region in a child.

Alopecia, palmoplantar keratoderma, skin fragility and follicular hyperkeratoses due to compound heterozygous mutations in desmoplakin.

Inherited mutations in desmosome genes can present with a spectrum of skin, hair and cardiac abnormalities. Here we describe a 4‐year‐old Turkish boy with a cardio‐cutaneous syndrome resulting from compound heterozygous nonsense mutations in desmoplakin. Early recognition of such cases by clinical awareness of the dermatological features and molecular diagnostics can improve patient management through early cardiac support, although the risk of cardiomyopathy and arrhythmias poses a major health concern.

KID syndrome patient with toe walking: A case report

Objective: To report on a 10-year-old boy with KID syndrome who presented with a new onset unilateral toe walking. Method: This patients equinus contracture was evaluated clinically. The patient underwent to surgical lengthening of Achilles tendon. After the surgery, the patient was placed in a short-leg walking cast for 6 weeks and then received a rehabilitation programme involving stretching exercises. Results: Successful heel-to-toe gait was achieved in 3 weeks following surgery and rehabilitation. Conclusion: Surgical intervention and postsurgical rehabilitation may be indicated for children who present with toe walking attributed to contracture in the Achilles tendon.