A case of Papular Mucinosis Associated with Ocular Findings and Responded to Treatment with Cyclophosphamide

G�lcan, P�nar; Ko�, Melek; Baran, Fehmi; Kocaba�o�lu, Cemal; Mudun, A. Baki

A case of Papular Mucinosis Associated with Ocular Findings and Responded to Treatment with Cyclophosphamide [Turkderm-Turk Arch Dermatol Venereol]

Turkderm-Turk Arch Dermatol Venereol. 2006; 40(B): 88-92

A case of Papular Mucinosis Associated with Ocular Findings and Responded to Treatment with Cyclophosphamide

P�nar G�lcan¹, Melek Ko�¹, Fehmi Baran¹, Cemal Kocaba�o�lu², A. Baki Mudun³
¹Sa�l�k Bakanl�� Okmeydan� E�itim Ve Ara�t�rma Hastanesi Dermatoloji Klini�i, �stanbul, Turkey
²Sa�l�k Bakanl�� Okmeydan� E�itim Ve Ara�t�rma Hastanesi Fizik Tedavi Ve Rehabilitasyon Klini�i, �stanbul, Turkey
³Sa�l�k Bakanl�� Okmeydan� E�itim Ve Ara�t�rma Hastanesi G�z Hastal�klar� Klini�i, �stanbul, Turkey

Papular mucinosis or lichen myxedematosus is a rare disorder characterized by proliferation of fibroblasts and excessive deposition of acid mucopolysaccharides in the skin. A clinical variant of papular mucinosis is scleromyxedema, in which the disease is more generalized and is accompanied by erythema and sclerosis. Paraproteinaemia is almost always found on serum electrophoresis, particularly in those with the clinical form of scleromyxedema, although total serum protein values are usually normal. Ophtalmic findings in patients with scleromyxedema are corneal opacities, thickened eyelid skin, lagophthalmos, and ectropion. Despite experience with multiple forms of therapy, no single agent has been proved uniformly beneficial in this disorder.
A 54-year-old female patient who was diagnosed as scleromyxedema on the basis of clinic and histopathologic findings and whose skin and ocular disorder were both treated with cyclophosphamid is presented.

Keywords: Papular mucinosis, scleromyxedema, cyclophosphamid

G�z Bulgular� ile Birliktelik G�steren ve Siklofosfamid Tedavisine Yan�t Veren Bir Pap�ler M�sinoz Olgusu

P�nar G�lcan¹, Melek Ko�¹, Fehmi Baran¹, Cemal Kocaba�o�lu², A. Baki Mudun³
¹Sa�l�k Bakanl�� Okmeydan� E�itim Ve Ara�t�rma Hastanesi Deri ve Z�hrevi Hastal�klar Klini�i, �stanbul, T�rkiye
²Sa�l�k Bakanl�� Okmeydan� E�itim Ve Ara�t�rma Hastanesi Fizik Tedavi Ve Rehabilitasyon Klini�i, �stanbul, T�rkiye
³Sa�l�k Bakanl�� Okmeydan� E�itim Ve Ara�t�rma Hastanesi G�z Hastal�klar� Klini�i, �stanbul, T�rkiye

Pap�ler m�sinozis veya liken miks�dematozis, deride fibroblast proliferasyonu ve asit mukopolisakkaritlerin a��r� birikimi ile karakterize, nadir g�r�len bir hastal�kt�r. Skleromiks�dem hastal��n daha jeneralize oldu�u ve pap�ler lezyonlara diff�z skleroz ile eritemin de e�lik etti�i formdur. Hastalarda total serum protein de�erleri genellikle normal olmakla birlikte, �zellikle de skleromiks�dem klinik formunda serum protein elektroforezinde paraproteinemi hemen daima mevcuttur. Skleromiks�demli hastalarda saptanan g�z bulgular� korneal opasiteler, g�z kapa�� derisinde kal�nla�ma, lagoftalmi, ektropion olarak bildirilmi�tir. Hastal��n tedavisinde de�i�ik tedavi �emalar� denenmekle birlikte, tek ba��na faydal� bir ila� mevcut de�ildir.
Paraproteineminin e�lik etmedi�i, fakat g�z muayenesinde bilateral yo�un papilla ve makula �demi ile periferik retinada noktasal hiperfl�resan g�steren lezyonlar �eklinde m�sin�z birikimler saptanan, klinik ve histopatolojik �zellikleri ile skleromiks�dem tan�s� koyulan ve siklofosfamid ile tedavi edilen 54 ya��nda bir bayan hasta sunulmaktad�r.

Anahtar Kelimeler: Pap�ler m�sinoz, skleromiks�dem, siklofosfamid

P�nar G�lcan, Melek Ko�, Fehmi Baran, Cemal Kocaba�o�lu, A. Baki Mudun. A case of Papular Mucinosis Associated with Ocular Findings and Responded to Treatment with Cyclophosphamide. Turkderm-Turk Arch Dermatol Venereol. 2006; 40(B): 88-92

Corresponding Author: P�nar G�lcan, T�rkiye

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