Ekrem AKTAŞ, Pınar KULLUK, Serap UTAŞ, Servet KESİM

Asitretin ile tedavi edilen bir Mal de Meleda Olgusu

Mal de Meleda (MdM) nadir görülen otozomal resesif geçiş gösteren bir deri hastalığıdır. 'Eldiven ve çorap' tarzı dağılım gösteren palmoplantar keratoderma (PPK), keratotik cilt lezyonları, perioral eritem, brakidaktili ve tırnak bozuklukları ile karakterizedir. Oral retinoidlerin keratinizasyon bozukluklarındaki etkisi iyi bilinmektedir. Burada her iki el içi ve ayak tabanında kalınlaşma şikayeti olan ve sistemik asitretin ile tedavi edilen 69 yaşında bir MdM olgusu sunulmaktadır.

A case of Mal de Meleda treated with acitretin

Mal de Meleda is a rare autosomal recessive skin disorder characterized by palmoplantar keratoderma with 'glove and sock' distribution, keratotic skin lesions, perioral erythema, brachydactyly and nail abnormalities. The efficacy of the oral retinoids in disorders of keratinization is well established. Herein, we present a 69 years old patient complainig about thickening of the skin of his palms and soles and diagnosed as Mal de Meleda and treated with acitretin.

PDF

___

1. Patel H, Nardelli M, Fenn T, et al. Homozigoty at chromosome 8qter in individuals affected by Mal de Meleda (Meleda disease) orginating from the island of Meleda. Br J Dermatol 2001; 144: 731–734
2. Bouadjar B, Benmazouzia S, Prud’homme JF. Clinical and genetic studies of 3 large, consanguineous, Algerian families with Mal de Meleda. Arc Dermatol 2000; 136: 1247–1252.
3. Fischer J, Boudjar B, Heilig R, et al. Mutations in the gene encoding SLURP–1 in Mal de Meleda. Hum Mol Genet 2001; 10: 875-880.
4. Yerebakan O, Hu G, Yılmaz E, Çelebi JT. A novel mutation in the ARS (component b) gene encoding SLURP–1 in a family with Mal de Meleda. Clin and Exp Dermatol 2003; 28: 542–544.
5. Ergin Ş, Şanlı Erdoğan B, Aktan Ş. Mal de Meleda: a new geographical localization in Anatolia. Dermatology 2003; 206: 124–130.
6. van de Kerkhof PCM, van Dooren-Greebe RJ, Steijlen PM. Acitretin in the treatment of Mal de Meleda. Br J Dermatol 1992;127:191–192.
7. Tüzün Y. Genodermatozlar. Dermatoloji. Ed. Tüzün Y, Kotoğyan A, Aydemir EH, Baransu O. 2. baskı. İstanbul, Nobel Tıp Kitapevi, 1994; 582.
8. Patel S, Davidson LE. Papillon- Lefévre Syndrome: a report of two cases. Int J Pediatr Dent 2004; 14: 288–294.
9. Braun-Falco O, Plewig G, Wolf HH, Burgdorf WHC. Dermatology. 2th ed. Berlin, Springer-Verlag, 2000; 727–729.
10. Capella GL, Fracchiolla C, Frigerio E, Altomare G. A controlled study of comparative efficacy of oral retinoids and topikal betametasone / salisylic acid for chronic hyperkeratotic palmoplantar dermatitis. J Dermatolog Treat 2004; 15: 88–93.
11. Reed ML, Stanley J, Stengel F, Shupack JL, Benjamin DM. Mal de Meleda treated with 13-cis retinoic acid. Arc Dermatol 2000; 136: 1247–1252.
12. Kaskel P, Leiter U, Krähn G, Schiner R, et al. PUVA-bath photochemotherapy for congenital palmoplantar keratoderma in an 11-year-old girl. Br J Dermatol 2000; 143: 464–465.
13. Brambilla L, Pigatto PD, Boneschi V, Altomare GF, Finzi AF. Unusual cases of Meleda keratoderma treated with aromatic retinoid etretinate. Dermatologica 1984; 168: 283–286.
14. Lienemann AO, Colucci VJ, Jones MS, Trauscht JM. Treatment of palmoplantar keratoderma with continuous infusion 5-fluorouracil. Cutis 2004; 73: 303–308
15. Happle R, van de Kerkhof PC, Traupe H. Retinoids in disorders of keratinization: their use in adults. Dermatologica 1987; 175 Suppl 1: 107–124.
16. Thestrup-Pedersen K, Andersen KE, Menne T, Veien NK. Treatment of hyperkeratotic dermatitis of the palms (ec-zema keratoticum) with oral acitretin. A single-blind placebo-controlled study. Acta Derm Venereol 2001; 81: 353–355.
17. Lee MR, F Wong LC, Fischer GO. Papillon-Lefévre syndrome treated with acitretin. Aust J Dermatol 2005; 46: 199–201.