A case of familial lichen amyloidosis/Ailesel liken amiloidoz olgusu

• Published in: Türkderm p. 137
• Main Authors: Ergin, Seniz, Demirkan, Nese, Kacar, Nida, Erdogan, Berna Sanli, Akman, Hatice
• Format: Journal Article
• Language: Turkish
• Published: Galenos Yayinevi Tic. Ltd 01-12-2008
• Subjects: Amyloidosis; Care and treatment; Case studies; Diagnosis; Patient outcomes; Risk factors; Turkey
• ISSN: 1019-214X

Familial lichen amyloidosis which is also referred to familial primary cutaneous amyloidosis is a rare clinical variant of cutaneous amyloidosis. Lichen amyloidosis is characterized by persistent, pruritic, small brown papules often located on anterior surfaces of legs which show tendency to form plaques. Amyloid deposits would be identified in papillary dermis in histopathological examination. In our clinic, a 42 year old woman with a widespread involvement describing that similar skin findings were present in her both daughters, elder brother and her nephew was evaluated with suspicion of lichen amyloidosis. In histopathological examination of the involved skin, because of determining amyloid deposits in papillary dermis the case was cited as lichen amyloidosis. Our case was searched for the accompanying diseases such as atopic dermatitis, chronic urticaria, lichen planus, multiple endocrine neoplasia and Kimura disease. The family history of our patient was consistent with autosomal dominant inheritance. Familial lichen amyloidosis has been reported as cases with autosomal dominant inheritance from Russia, Germany, United Kingdom and South America. The genetic researches over familial lichen amylodiosis are limited to the cases with multiple endocrine neoplasia. In this rarely reported cases, further genetical researches are necessary in order to determine the responsible gen locus. (Turkderm 2008; 42: 137-9) Key Words: Familial lichen amyloidosis Ailesel primer kutanoz amiloidoz olarak da adlandirilan ailesel liken amiloidoz, primer kutanoz amiloidozun nadir gorulen bir klinik varyantidir. Liken amiloidoz, genellikle bacak on yuzunde yerlesimli, birleserek plak yapma egilimi gosteren, inatci pruritik kucuk kahverengi papuller ile karakterizedir. Histopatolojik incelemede papiller dermiste amiloid birikimi izlenir. Klinigimizde, vucutta yaygin tutulumun izlendigi, iki kizinda, erkek kardesi ve kardesinin oglunda da benzer yakinmalar oldugunu tanimlayan 42 yasinda kadin olgu liken amiloidoz on tanisi ile degerlendirildi. Lezyonlu deriden alinan biyopsi orneginin histopatolojik incelemesinde, papiller dermiste amiloid birikimi saptanmasi uzerine liken amiloidoz tanisi konuldu. Olgumuz atopik dermatit, kronik urtiker, liken planus, multipl endokrin neoplazi ve Kimura hastaligi gibi eslik eden hastaliklar yonunden arastirildi. Olgumuzun aile oykusu, otozomal dominant kalitim ile uyumluydu. Ailesel liken amiloidoz, Rusya, Almanya, Ingiltere ve Guney Amerika'da otozomal dominant kalitimin goruldugu olgular seklinde bildirilmistir. Ailesel liken amiloidoz ile ilgili genetik arastirmalar multipl endokrin neoplazi ile birliktelik gosteren olgularla sinirlidir. Literaturde nadir bildirilen bu olgularda sorumlu gen lokusunun belirlenmesi icin ileri genetik arastirmalara gereksinim vardir. (Turkderm 2008; 42: 137-9) Anahtar Kelimeler: Ailesel liken amiloidoz