Discrete Papular Mucinosis-A Rare Subtype of Lichen Myxoedematosus

Kaya Ak��, Havva; Eskio�lu, Fatma; �zt�rk, Evrim

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Discrete Papular Mucinosis-A Rare Subtype of Lichen Myxoedematosus [Turkderm-Turk Arch Dermatol Venereol]

Turkderm-Turk Arch Dermatol Venereol. 2011; 45(2): 104-106 | DOI: 10.4274/turkderm.45.24

Discrete Papular Mucinosis-A Rare Subtype of Lichen Myxoedematosus

Havva Kaya Ak��¹, Fatma Eskio�lu¹, Evrim �zt�rk²
¹Department Of Dermatology, D��kap� Y�ld�r�m Beyaz�t Education And Training Hospital, Ankara, Turkey
²Department Of Pathology, D��kap� Y�ld�r�m Beyaz�t Education And Training Hospital, Ankara, Turkey

Lichen myxoedematosus (synonym, papular mucinosis) is an uncommon, chronic, idiopathic disorder characterized by lichenoid papules, nodules and/or plaques due to dermal mucin deposition and a variable degree of fibrosis in the absence of thyroid dysfunction. Actually, lichen myxoedematosus includes two clinicopathologic subsets: a generalized papular and
sclerodermoid form (also called scleromyxedema) with a monoclonal gammopathy and systemic, even lethal, manifestations and a localized papular form with non-disabling course. Discrete papular mucinosis is a rare subtype of the localized form and can be associated with hepatitis C virus and human immunodeficiency virus (HIV) infection. Only 12 cases unrelated to HCV or HIV infection have been described in the literature to date. Herein, we report a 64-year-old woman who presented with asymptomatic, flat, flesh-coloured papules on her neck, upper trunk and proximal extremities. A skin biopsy from a papule on her neck demonstrated dermal mucin deposition after alcian blue staining. The number of fibroblasts was increased. Laboratory studies revealed normal thyroid function tests. Serum protein electrophoresis did not show any evidence of
a monoclonal gammopathy. Serology tests for HCV and HIV were negative.

Keywords: Papular mucinosis, scleromyxedema

"Diskret" Pap�ler M�sinoz: Liken Miks�dematozun Nadir Bir Subtipi

Havva Kaya Ak��¹, Fatma Eskio�lu¹, Evrim �zt�rk²
¹D��kap� Y�ld�r�m Beyaz�t E�itim Ve Ara�t�rma Hastanesi, Deri Ve Z�hrevi Hastal�klar Klini�i, Ankara
²D��kap� Y�ld�r�m Beyaz�t E�itim Ve Ara�t�rma Hastanesi, Patoloji Klini�i, Ankara

Liken miks�dematoz (pap�ler m�sinoz) tiroid hastal�� olmaks�z�n dermal m�sin birikimi ve fibrozise ba�l� olarak geli�en likenoid pap�l, nod�l ve/veya plaklar ile karakterize s�k g�r�lmeyen, kronik, idiyopatik bir hastal�kt�r.Liken miks�dematoz klinikopatolojik olarak iki subgrup i�erir: monoklonal gammopati ile beraber sistemik, hatta letal bulgular� olan generalize pap�ler ve sklerodermoid form (skleromiks�dem de denir) ve daha selim prognozlu lokalize pap�ler form."Discrete" pap�ler m�sinoz, Hepatit C vir�s (HCV) ve insan imm�nyetmezlik vir�s (HIV) infeksiyonu ile ili�kili olabilen lokalize formun nadir bir subtipidir.Bug�ne kadar literat�rde HCV veya HIV infeksiyonu ile ili�kisi olmayan sadece 12 olgu bildirimi vard�r. Burada v�cu-dunda �ok say�da, asemptomatik, deri renginde pap�lleri olan, histopatolojik incelemede dermal m�sin birikimi tespit edilen, tiroid hastal�� ve monoklonal gammopati saptanmayan, viral belirleyicileri negatif olan 64 ya��nda kad�n hastay� sunuyoruz.

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

Havva Kaya Ak��, Fatma Eskio�lu, Evrim �zt�rk. Discrete Papular Mucinosis-A Rare Subtype of Lichen Myxoedematosus. Turkderm-Turk Arch Dermatol Venereol. 2011; 45(2): 104-106

Corresponding Author: Havva Kaya Ak��, T�rkiye

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