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Lameller İktiyoz: Olgu Sunumu

Year 2010, Volume: 8 Issue: 1, 154 - 157, 01.06.2010

Abstract

Giriş: İktiyoziform dermatozlar deri yüzeyinde aşırı skuam birikimiyle karakterize bir grupkalıtsal hastalıktır. Lameller iktiyoz otozomal resesif olarak aktarılan bir iktiyoz türüdür.Doğumla birlikte başlayan hastalık zamanla klinik olarak daha belirgin hale gelir ve tümderi yüzeyini kaplayan gri-kahverengi skuamlar gözlenir. Ektropiyon, palmoplantarhiperkeratoz, skatrisyel alopesi ve hipohidroz diğer eşlik eden bulgulardır. Topikal olaraknemlendiriciler, salisilik veya glikolik asit içeren keratolitikler kullanılır. Sistemik asitretinkullanımı ile semptomlar büyük ölçüde düzeltilebilir. Olgu Sunumu: Lameller iktiyoz tanılı 5 yaşında kız olgu sunulmaktadır. Kollodyon membraniçinde doğan hastada derideki skuamların yanı sıra ektropiyon, kulaklarda ve tırnaklardadeformiteler mevcuttu. Günde 10 mg asitretin ile oldukça iyi klinik düzelme elde edildi. Tartışma: Lameller iktiyoz şiddetli bir iktiyoz formudur, topikal kullanılan ilaçlar yetersizkalabilir. Sistemik asitretin tedavisi semptomları büyük ölçüde düzeltebilir ancak uzundönem kullanımda gelişebilecek yan etkiler kullanımı kısıtlamaktadır

References

  • 1. Odom RB, James WD, Berger TG. Andrews’ Diseases of the Skin. 9th ed. Philadelphia: WB Saunders Co; 2000. p. 682-732.
  • 2. ‹kizo¤lu G. Genodermatozlar. In: Dermatoloji. Tüzün Y, Gürer MA, Serdaro¤lu S, O¤uz O, Aksungur VL (eds). 3. bask› ‹stanbul: Nobel T›p; 2008. p. 1609-736.
  • 3. Richard G. Molecular genetics of the ichthyoses. Am J Med Genet C Semin Med Genet 2004; 15;131:32-44.
  • 4. Vahlquist A, Ganemo A, Virtanen M. Congenital ichthyosis. An overview of current and emerging therapies. Acta Derm Venereol 2008; 88:4-14.
  • 5. Göçmen I, Mete Z, Çolak S. Lameller iktiyoz, olgu sunumu. Türkiye Klinikleri J Dermatol 1993; 3:28-31.
  • 6. Hofmann B, Stege H, Ruzicka T, Lehmann P. Effect of topical tazarotene in the treatment of congenital ichthyoses. Br J Dermatol 1999; 141:642-6.
  • 7. Kundu RV, Garg A, Worobec SM. Lamellar ichthyosis treated with tazarotene 0,1% gel. J Am Acad Dermatol 2006; 55 (5 Suppl): S94-5.
  • 8. Lucker GP, van de Kerkhof PC, van Dïjk MR, Steijlen PM. Effect of topical calcipotriol on congenital ichthyoses. Br J Dermatol 1994; 131:546-50.
  • 9. Redondo P, Bauzá A. Topical N-acetylcysteine for lamellar ichthyosis. Lancet 1999; 354:1880.
  • 0. DiGiovanna JJ, Robinson-Bostom L. Ichthyosis: etiology, diagnosis, and management. Am J Clin Dermatol 2003; 4:81-95.
  • 11. Oji V, Traupe H. Ichthyosis: clinical manifestations and practical treatment options. Am J Clin Dermatol 2009; 10:351-64.
  • 12. Verfaille CJ, Borgers M, Steensel MA. Retinoic acid metabolism blocking agents (RAMBAs): a new paradigm in the treatment of hyperkeratotic disorders. J Dtsch Dermatol Ges 2008; 6:355-64.
  • 13. Verfaille CJ, Vanhautte FP, Vardon CB, Steensel MA, Steijlen PM. Oral liarozole vs. acitretin in the treatment of ichthyosis: a phase II/III multicentre, double-blind, randomized, active-controlled study. Br J Dermatol 2007; 156:965-73.
  • 14. Haenssle HA, Finkenrath A, Hausser I, Oji V, Traupe H, Hennies HC, et al. Effective treatment of severe thermodysregulation by oral retinoids in a patient with recessive congenital lamellar ichthyosis. Clin Exp Dermatol 2008; 33:578-81.
  • 15. Özkan AS, Alper S. Sistemik tedavi. Dermatoloji’de. Tüzün Y, Gürer MA, Serdaro¤lu S, O¤uz O, Aksungur VL, editörler. 3. bask›. ‹stanbul: Nobel T›p; 2008. p. 2147-258.
  • 16. Katugampola RP, Finlay AY. Oral retinoid therapy for disorders of keratinization: single-centre retrospective 25 years’ experience on 23 patients. Br J Dermatol 2006; 154:267-76.
  • 17. Zhang X, He Y, Zhou H, Luo Q, Li C. Severe ichthyosis-related disorders in children: Response to acitretin. J Dermatol Treatment 2007; 18:118-22.

Lamellar Ichtyosis: Case Report

Year 2010, Volume: 8 Issue: 1, 154 - 157, 01.06.2010

Abstract

Introduction: Ichtyosiform dermatoses are a group of hereditary disorders characterizedby excessive scaling on the skin. Lamellar ichtyosis is an autosomal recessive disorder.The clinical findings are seen at birth and become widespread and prominent in time;gray-brown scales are seen all over the body. Emollients and keratolytics containing salicylic acid or glycolic acid are used topically. Symptoms are improved significantly byacitretin usage. Case Report: Here we present a 5-year-old girl with lamellar ichtyosis. She was born ina collodion membrane. Besides the skin scales, ectropion and deformities of the nailsand ears were observed. Significant clinical improvement was seen with acitretin 10 mg/day therapy. Conclusion: Lamellar ichtyosis is a severe form of ichtyosiform dermatoses and topicalagents may be insufficient. Systemic acitretin treatment improves the symptoms significantly but long term side effects limit its usage

References

  • 1. Odom RB, James WD, Berger TG. Andrews’ Diseases of the Skin. 9th ed. Philadelphia: WB Saunders Co; 2000. p. 682-732.
  • 2. ‹kizo¤lu G. Genodermatozlar. In: Dermatoloji. Tüzün Y, Gürer MA, Serdaro¤lu S, O¤uz O, Aksungur VL (eds). 3. bask› ‹stanbul: Nobel T›p; 2008. p. 1609-736.
  • 3. Richard G. Molecular genetics of the ichthyoses. Am J Med Genet C Semin Med Genet 2004; 15;131:32-44.
  • 4. Vahlquist A, Ganemo A, Virtanen M. Congenital ichthyosis. An overview of current and emerging therapies. Acta Derm Venereol 2008; 88:4-14.
  • 5. Göçmen I, Mete Z, Çolak S. Lameller iktiyoz, olgu sunumu. Türkiye Klinikleri J Dermatol 1993; 3:28-31.
  • 6. Hofmann B, Stege H, Ruzicka T, Lehmann P. Effect of topical tazarotene in the treatment of congenital ichthyoses. Br J Dermatol 1999; 141:642-6.
  • 7. Kundu RV, Garg A, Worobec SM. Lamellar ichthyosis treated with tazarotene 0,1% gel. J Am Acad Dermatol 2006; 55 (5 Suppl): S94-5.
  • 8. Lucker GP, van de Kerkhof PC, van Dïjk MR, Steijlen PM. Effect of topical calcipotriol on congenital ichthyoses. Br J Dermatol 1994; 131:546-50.
  • 9. Redondo P, Bauzá A. Topical N-acetylcysteine for lamellar ichthyosis. Lancet 1999; 354:1880.
  • 0. DiGiovanna JJ, Robinson-Bostom L. Ichthyosis: etiology, diagnosis, and management. Am J Clin Dermatol 2003; 4:81-95.
  • 11. Oji V, Traupe H. Ichthyosis: clinical manifestations and practical treatment options. Am J Clin Dermatol 2009; 10:351-64.
  • 12. Verfaille CJ, Borgers M, Steensel MA. Retinoic acid metabolism blocking agents (RAMBAs): a new paradigm in the treatment of hyperkeratotic disorders. J Dtsch Dermatol Ges 2008; 6:355-64.
  • 13. Verfaille CJ, Vanhautte FP, Vardon CB, Steensel MA, Steijlen PM. Oral liarozole vs. acitretin in the treatment of ichthyosis: a phase II/III multicentre, double-blind, randomized, active-controlled study. Br J Dermatol 2007; 156:965-73.
  • 14. Haenssle HA, Finkenrath A, Hausser I, Oji V, Traupe H, Hennies HC, et al. Effective treatment of severe thermodysregulation by oral retinoids in a patient with recessive congenital lamellar ichthyosis. Clin Exp Dermatol 2008; 33:578-81.
  • 15. Özkan AS, Alper S. Sistemik tedavi. Dermatoloji’de. Tüzün Y, Gürer MA, Serdaro¤lu S, O¤uz O, Aksungur VL, editörler. 3. bask›. ‹stanbul: Nobel T›p; 2008. p. 2147-258.
  • 16. Katugampola RP, Finlay AY. Oral retinoid therapy for disorders of keratinization: single-centre retrospective 25 years’ experience on 23 patients. Br J Dermatol 2006; 154:267-76.
  • 17. Zhang X, He Y, Zhou H, Luo Q, Li C. Severe ichthyosis-related disorders in children: Response to acitretin. J Dermatol Treatment 2007; 18:118-22.
There are 17 citations in total.

Details

Primary Language Turkish
Journal Section Case Report
Authors

Kamer Gündüz

Tuba Çelebi Kayhan This is me

Gülsüm Gençoğlan This is me

İşıl İnanır This is me

Peyker Temiz This is me

Publication Date June 1, 2010
Published in Issue Year 2010 Volume: 8 Issue: 1

Cite

APA Gündüz, K., Kayhan, T. Ç., Gençoğlan, G., İnanır, İ., et al. (2010). Lameller İktiyoz: Olgu Sunumu. Güncel Pediatri, 8(1), 154-157.
AMA Gündüz K, Kayhan TÇ, Gençoğlan G, İnanır İ, Temiz P. Lameller İktiyoz: Olgu Sunumu. Güncel Pediatri. June 2010;8(1):154-157.
Chicago Gündüz, Kamer, Tuba Çelebi Kayhan, Gülsüm Gençoğlan, İşıl İnanır, and Peyker Temiz. “Lameller İktiyoz: Olgu Sunumu”. Güncel Pediatri 8, no. 1 (June 2010): 154-57.
EndNote Gündüz K, Kayhan TÇ, Gençoğlan G, İnanır İ, Temiz P (June 1, 2010) Lameller İktiyoz: Olgu Sunumu. Güncel Pediatri 8 1 154–157.
IEEE K. Gündüz, T. Ç. Kayhan, G. Gençoğlan, İ. İnanır, and P. Temiz, “Lameller İktiyoz: Olgu Sunumu”, Güncel Pediatri, vol. 8, no. 1, pp. 154–157, 2010.
ISNAD Gündüz, Kamer et al. “Lameller İktiyoz: Olgu Sunumu”. Güncel Pediatri 8/1 (June 2010), 154-157.
JAMA Gündüz K, Kayhan TÇ, Gençoğlan G, İnanır İ, Temiz P. Lameller İktiyoz: Olgu Sunumu. Güncel Pediatri. 2010;8:154–157.
MLA Gündüz, Kamer et al. “Lameller İktiyoz: Olgu Sunumu”. Güncel Pediatri, vol. 8, no. 1, 2010, pp. 154-7.
Vancouver Gündüz K, Kayhan TÇ, Gençoğlan G, İnanır İ, Temiz P. Lameller İktiyoz: Olgu Sunumu. Güncel Pediatri. 2010;8(1):154-7.